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Systemic Capillary Leak Syndrome

What is Systemic Capillary Leak Syndrome?

Systemic Capillary Leak Syndrome (SCLS) is an exceedingly rare, life- and limb-threatening disorder characterized by acute and severe recurrent attacks featuring a rapid fall in blood pressure due to the temporary leak of plasma out of the blood circulatory system.

This virtual community is dedicated to the memory of Judith (Judy) Lynne Davis (1958-2009) (judithdavis3), one of its founding members and a victim of a very severe episode of SCLS that took her life in November 2009.

We also mourn the death of nine other SCLS patient members of this community: Mario Gatto (mariogatto) from Naples, Italy, who passed away in December 2009; Denise Weston (mdweston) from Ohio, USA, who passed on March 2011; Bruno Galien (bruno) from Nord-Pas-de-Calais, France, who passed away in February 2012; Guy Allen Overland (allenoverland) from the Washington DC area, USA, who passed on January 2015; Marilyn Meaux (maire602) from Louisiana, USA, who passed away in March 2017; Julie Eady (jodono) from Perth, Australia, who passed on September 2017; Cara O'Hagan (Cara) from Dublin, Ireland, who passed away in February 2018; David Colburn (davec) from Gainesville, Florida, who passed on in September 2019; and Jeff Isenhour (jisenhour) from Wahington, DC, who died in September 2021.

 

Synonyms

  • Clarkson or Clarkson's Disease

Systemic Capillary Leak Syndrome (SCLS) is an exceedingly rare, life- and limb-threatening disorder characterized by acute and severe recurrent attacks featuring a rapid fall in blood pressure due to the temporary leak of plasma out of the blood circulatory system.

This virtual community is dedicated to the memory of Judith (Judy) Lynne Davis (1958-2009) (judithdavis3), one of its founding members and a victim of a very severe episode of SCLS that took her life in November 2009.

We also mourn the death of nine other SCLS patient members of this community: Mario Gatto (mariogatto) from Naples, Italy, who passed away in December 2009; Denise Weston (mdweston) from Ohio, USA, who passed on March 2011; Bruno Galien (bruno) from Nord-Pas-de-Calais, France, who passed away in February 2012; Guy Allen Overland (allenoverland) from the Washington DC area, USA, who passed on January 2015; Marilyn Meaux (maire602) from Louisiana, USA, who passed away in March 2017; Julie Eady (jodono) from Perth, Australia, who passed on September 2017; Cara O'Hagan (Cara) from Dublin, Ireland, who passed away in February 2018; David Colburn (davec) from Gainesville, Florida, who passed on in September 2019; and Jeff Isenhour (jisenhour) from Wahington, DC, who died in September 2021.

Acknowledgement of Systemic Capillary Leak Syndrome has not been added yet.

Less than one in 1 million people are affected by this disease. The onset of SCLS will usually occur in adults. However, SCLS can affect people of all ages.

Name Abbreviation
Clarkson or Clarkson's Disease Clarkson

Systemic Capillary Leak Syndrome (SCLS) is idiopathic. Thus, at present, there are no known causes. Probably a mid-life gene mutation takes place that renders those affected vulnerable. Possibly immune-deficient in some way to these curiously self-reversing capillary leaks.

Many patients report having a runny nose, flu-like symptoms, gastro-intestinal disorders, a general weakness or pain in their limbs, swelling in the face or hands and feet, or very cold hands and feet, but others get no particular or consistent warning signs.

Name Description
Swelling swelling
Myalgia Myalgia is muscle pain
Rhinorrhea Rhinorrhea is a runny nose
Dizziness Dizziness
Lightheadedness Lightheadedness
Hypotension Hypotension is abnormally low blood pressure
Hemoconcentration Hemoconcentration is the decrease of the fluid content of the blood, with increased concentration of formed elements
Hypoalbuminemia Hypoalbuminemia is low levels of protein in the blood
Nausea Nausea
Excessive thirst Excessive thirst
Generalized edema Generalized edema
Decline in clinical picture Clinical picture declines rapidly within hours
Cold limbs and sweating Cold limbs and sweating
Rapid swelling and compartment syndrome Rapid swelling of all limbs with development of compartment syndrome, especially during IV fluid administration
Decreased urine output Decreased urine output
Vomiting Vomiting
Intestinal cramps Cramps
Diarrhea Diarrhea
Fatigue Fatigue
Headache Headache

The diagnosis of SCLS is made partly by exclusion, namely, by eliminating the possibility of other more common diseases, and is based on measurable, clinical symptoms such as hypotension or low blood pressure, hemoconcentration or decrease in blood volume, hypoalbuminemia or an abnormally low levels of a protein called albumin in urine, and the presence of a protein called Monoclonal Gammopathy of Unknown Significance (MGUS).

Some diagnostic tests may include blood and urine tests to check for abnormalities such as dark urine, concentrated blood, or low serum albumin in the blood.

  • IVIG 1 g/kg twice (two infusions) as soon as possible after arrival at the Emergency Room.
  • Methylprednisolone 125 mg intravenously one time, then repeated as needed.
  • Judicious use of intravenous fluids (IVF) in boluses and drips to keep central venous pressure (CVP) above zero.
  • Phenylephrine or Norepinephrine for hypotension, early institution.
  • 50 ml of 25% albumin, then repeated as needed.
  • Continuous CVP monitoring, immediate (stat) and continuous (serial) lab work including createnine phosphokinase (CPK) and lactate.
  • Immediate orthopedics consult and compartment pressure measurement early on to prevent limb fasciotomies if compartment pressures or CPK are high. 
  • Venous doppler for deep vein thrombosis (DVT) prevention, may need full anticoagulation to prevent blood clotting.

Treatment of an episode of SCLS requires recognition that there are two phases. The first phase, which often lasts 24-72 hours, is called the leak phase.  A plasma and albumin leak from the capillaries into the tissue spaces causes swelling, especially in the muscle compartments of the extremities. The blood pressure falls and the red cells concentrate. This loss of fluid from the vascular system has similar effects on circulation as dehydration, slowing both the flow of oxygen carrying blood to tissues and the output of urine. Physicians should refrain from trying to stop or control the capillary leak except by administering IVIG at the outset, possibly supplemented by injected steroids. Even though blood pressure readings may reach and remain at very low levels, oliguria should be tolerated and aggressive intravenous fluid administration should be avoided, because fluids will mostly leak out and cause compartment syndrome and other complications.

The goal of saline and vasopressors administered should NOT be to restore a "normal" blood pressure (or urine flow), but to maintain it at a minimal level sufficient to avoid permanent damage to vital organs. Measurement of central venous or arterial pressure in an ICU setting is often necessary to achieve this delicate balance. When too much fluid is administered, the result is excessive swelling, and the patient will usually require surgical decompression of the limbs. In this procedure, known as a fasciotomy, the skin of the arms and/or legs is incised to release the compressive pressure the retained fluid is having on blood flow to and from the extremities.

The second phase of the treatment is known as the recruitment phase, when fluids and albumin are reabsorbed from the tissues during at least a couple of days. In this phase, the capillary leak has ended and the main threat is fluid overload. If intravenous fluids were given in excess, they usually cause an accumulation of fluid in the lungs and around other vital organs. Most of the patient deaths happen during this recruitment phase so it is important that diuretics be administered to help patients discharge all the fluid previously given -- and to keep them from backing up, especially into the lungs.

As concerns episode prevention, two approaches have been tried: high-dose β-agonists like theophylline and terbutaline, and a prophylaxis with high-dose intravenous immunoglobulin (IVIG) infusions. In the past two decades, most SCLS patients have been migrated from the former to the latter in Canada, Europe, the United States and beyond, because IVIG therapy leads to far superior results. IVIG infusions every 2 or 4 weeks prevent or minimize leak episodes far more effectively than any other therapy, and do not have the adverse side effects that treatment with high doses of β-agonists like theophylline and terbutaline entails.

The prognosis is uncertain and depends on (a) how well episodes are managed, in terms of preventing permanent damage to vital organs and extremities; and (b) the ability to prevent episodes altogether.

There are two main treatments to prevent episodes of SCLS. The oldest is the Mayo Clinic’s approach of a preventive therapy with theophylline (or aminophylline) and terbutaline tablets taken on a daily basis. However, these medications, meant to reduce endothelial hyperpermeability, have very unpleasant side effects, and often prove ineffective, providing partial and transient improvement.

The newest is the French preventive regimen, which involves monthly infusions of immunoglobulins (IVIG). By now there is ample evidence that IVIG (usually, 1 or 2 gr/kg per month, administered over two consecutive days) has worked for many patients in Europe, the United States, and beyond for over 15 years, thus having become the gold standard of preventive care.

Name Description
Medical help

Find yourself a compassionate physician, preferably a specialist in internal medicine or hematology affiliated with a major university hospital, willing to do his/her homework on this rare disorder (namely, read the literature and follow the instructions), and willing to consult with the few SCLS experts available:

In the United States,
Dr. Mark S. Pecker
Professor of Clinical Medicine
Weill Cornell Medical College
New York, NY
tel. 1646-962-2605
email mpecker@med.cornell.edu

In Europe:
Prof. Dr. Zahir Amoura
Département de Médecine Interne
Hôpital de la Pitié-Salpêtrière, 47-83 Bd. de l'Hôpital, 75013 Paris
tél. 0033 1 4217 8001
email zahir.amoura@aphp.fr

Clinical Study Volunteer
Patients who have been diagnosed as having SCLS and who are at least 16 years old are wanted for participation in the only scientific study of the illness taking place anywhere in the world: at the National Institutes of Health in Bethesda, MD, right outside Washington DC. You must have a documented medical history including at least one acute episode of SCLS or else continuous symptoms of periodic hemoconcentration, hypotension and protein leakage. Have your primary doctor contact Ms. Robin Eisch at eischar@niaid.nih.gov, before sending in the requisite letter of referral with your medical history and laboratory studies to the lead clinical investigator, Dr. Kirk Druey, kdruey@niaid.nih.gov, tel. 301-435-8875. Once accepted into the clinical research study, you will be invited to come to NIH and spend about 4 days there for the purpose of being examined, donating blood, and being subjected to various tests (e.g., clinical digital photography of your blood vessels). Depending on circumstances, you probably will have time off to do sightseeing in the capital area during your stay at NIH.

 

Please see the Disorder Resources section.

SCLS and Meniere's Created by kevinl1970
Last updated 7 Jul 2022, 12:29 AM

Posted by kevinl1970
7 Jul 2022, 12:29 AM

Good afternoon, I was looking for a unique way to introduce myself to this fine community as I have also been recently diagnosed with SCLS.  Hopefully this is light at the end of the tunnel after five years of mystery attacks and hospitalizations. I've been told in the past I've possibly had septic shock, heart failure, kidney failure, severe dehydration, polycythemia vera, pneumonia, and many other things I cannot remember at the moment. About six months ago someone in the ER mentioned SCLS and when I read up on it, the symptoms, blood tests, etc. matched up closer than anything before. None of the dr's in my area (a major metropolitan city in the US) would agree to an evaluation, and I eventually ended up at the Mayo clinic. They ruled a few other things out, and here I am, now waiting for my first IVig treatment.

Being a rare disease, I have begun to start to question my diagnosis of Meniere's disease from 30 years ago. To date, I've had two otolaryngologists say "Yes it's Meniere's" and two say "It's definitely NOT Meniere's".  I have issues in both ears, one started when I was 18, the other three years later. The doctors say it's extremely rare to have it in both ears. So did I hit the jackpot of rare diseases or what? 

Some of the recent blood tests I've had indicate problems in my immune system, and one otolaryngologist mentioned it could be an autoimmune disorder causing the Meniere-like symptoms. 

So I guess once I get things stabilized with the SCLS I can re-evaluate on the Meniere's 

Has anyone here had anything like Meniere’s ?  i.e. sudden hearing loss, sudden vertigo, "drop attacks" where you fall due to loss of balance and can't stand up?

 

Fiance has SCLS after Covid shot and no Dr will help! Created by kage101
Last updated 5 Jul 2022, 06:45 PM

Posted by ValeriaSpain
5 Jul 2022, 06:44 PM

Hi Kage101,

I am familiar with SCLS episodes following a covid vaccine, especially tbe J&J one as we have had several cases here in Spain.

Furthermore, these cases are the main reason why my doctors advised me against getting vaccinated.

How is your fiancée coping with her symptoms? Were you able to attain medical assistance regarding her generalized oedema? 

 Kind regards,

Valeria.

Posted by kage101
5 Apr 2022, 02:46 AM

Thank you!

Posted by aporzeca
4 Mar 2022, 05:31 AM

kage101,

I'm so sorry to hear about your fiancée's medical problem!  While I'm not a physician, I've learned a great deal about SCLS during the past more than fifteen years, through this community and from personal experience, and in my opinion she does not suffer from SCLS.  

SCLS is an exceedingly rare episodic illness characterized by acute and severe attacks featuring a rapid fall in blood pressure due to the temporary leak of plasma out of the blood circulatory system.  Patients gain weight during an episode IF they are given replacement intravenous fluids, and then only during a few days.  Once the leak episode ends, and if they survive it, their body gets rid of all excess fluids, and thus their weight returns to normal.

In other words, SCLS features a self-reversing phenomenon.  Therefore, what you are describing, somebody gaining weight week after week, and month after month, is indicative of some other, chronic -- as opppsed to episodic -- swelling disorder.

If you can afford it, or can work out a deal with the hospital in advance, I suggest you make an appointment for her to be seen at the world-famous Cleveland Clinic.  People of means or with good insurance flock there from all over the country and even from abroad with all kinds of undiagnosed or unresolved health problems.

Failing that, your next-best bet (and closest option) is for her to be seen by an edema specialist at the Ohio State University Wexner Medical Center in Columbus, which is ranked #2 in Ohio.  This includes internists (big-picture clinicians), nephrologists (kidney specialists), and gastroenterologists (digestive tract or liver specialists), as well as bariatric (weight control) physicians.  Check out https://wexnermedical.osu.edu/find-a-doctor or else https://health.usnews.com/best-hospitals/area/oh/ohio-state-university-wexner-medical-center-6411100/doctors/internists

Good luck and keep us posted!

View Full Thread (1 more posts)
Covid-triggered episodes of SCLS? Created by aporzeca
Last updated 5 Jul 2022, 01:52 PM

Posted by ValeriaSpain
5 Jul 2022, 01:52 PM

Hi everyone! I hope you're all doing great. 

Even though this thread is about our experiences contracting Covid, I just wanted to share that I haven't caught it nor received the vaccine. 

I will revisit this thread and share my experience if that happens, however, I really hope I never do, especially when I'm not receiving IVIG infusions because my doctors say that "it's just a patch". I know its controversial, but so far they are currently looking for other ways to treat my chronic leakage.

They also think that it's not safe for me to get the vaccine as it would worsen endothelial hyperpermeability and escalate to a more serious episode. 

Best of health to all of you,

Valeria.

Posted by catsanmice
2 Jul 2022, 04:56 PM

Hi all - hope you are all well.

I didn't exactly have SCLS triggered by covid, but I definitely feel the vaccines triggered symptoms for me and that there was some effect of covid that seemed like SCLS.

I had 2 doses of the Pfizer vaccines in January and February of 2021. I had chronic symptoms of SCLS from that time until October 2021. I told my doctors I had gained weight prior to getting the vaccines and asked them to raise my dose of IViG to match my weight. We conferred with Dr. Druey and he agreed. Once my dose was raised, the chronic SCLS symptoms resolved.

I caught covid around 1/5/22, less than 2 weeks after an IViG infusion. I got over most symptoms in a week, but the exhaustion, lethargy and brain fog lasted until February. Those symptoms felt similar to some of the symptoms I get during an SCLS flare - very heavy, weak, dull, brain-foggy (translate that slang as "stupid"), and lethargic. I can't say I know covid would have been different for me if I didn't have SCLS, but I do kind of feel like that's the case. So no, I did not have a real SCLS flare due to covid, but maybe SCLS influenced how I experienced covid.

Because I had such a miserable time after the vaccines, I don't want to get boosters. I've made significant changes to my life in order to avoid having to do that.

My best wishes for us all, and thanks -

Cathy

 

Posted by catsanmice
2 Jul 2022, 04:43 PM

View Full Thread (6 more posts)
Vaccine Technology Vs. SCLS Created by Arielbatt
Last updated 26 Jun 2022, 05:49 PM

Posted by Arielbatt
26 Jun 2022, 05:49 PM

Thank you for your time.

Posted by aporzeca
26 Jun 2022, 01:23 PM

Ariel, I suggest you review two past discussion topics, https://rareshare.org/topics/2167 and https://rareshare.org/topics/2140, which have information relevant to your question.

Posted by Arielbatt
25 Jun 2022, 04:05 PM

Hi Arturo.  I will take the audacity to write in our mother tongue, and I will put the Google translation at the end.
 Many times I am left wondering how real they are, and I want to make sure that my query was well understood.
 I was not referring to the fact that the technology of the vaccines modifies the coverage capacity for Covid.  But to the possibility that the vaccine, by itself, has a greater possibility of triggering an attack both in the first dose and when changing technology.  I haven't seen a lot of SCLS attacks for the vaccine here on the forum... but I know there are.
 Perhaps because neither North America nor Europe recommend changing brands (is this everyone or just SCLS sufferers?) As always, thank you for your time and dedication.
 Ariel.

Hola Arturo. Me tomaré el atrevimiento de escribir en nuestra lengua madre, y pondré la traducción de Google al final. 
Muchas veces me queda la duda de que tan reales son, y quiero asegurarme de que mi consulta fue bien entendida.
No me refería a que la tecnología de las vacunas modifiquen la capacidad de cobertura al Covid. Sino al la posibilidad de que la vacuna,  por sí sola, tenga mayor posibilidad de desencadenar un ataque tanto sea en la primera dosis como al cambiar de tecnología. No he visto aquí en el foro, mucho de ataques SCLS por la vacuna... pero se que los hay. 
Quizas porque ni en América del Norte ni en Europa aconsejan el cambio de marca (esto es a todos o solo a enfermos de SCLS?) Como siempre, gracias por su tiempo y dedicación.
Ariel

View Full Thread (2 more posts)
Autonomic Dysfunction/Dysautonomia and SCLS Created by stedrick
Last updated 24 Apr 2022, 01:07 AM

Posted by aporzeca
24 Apr 2022, 01:07 AM

Susan,

I don't know and haven't heard anything about it, so I suggest you write to and ask Dr. Kirk Druey at NIH, kdruey@nih.gov

Posted by stedrick
23 Apr 2022, 11:56 AM

So far, I've only found one study, conducted in Italy, that postulates that chronic dysautonomia may trigger SCLS flares in addition to SCLS triggering temporary autonomic dysfunction.

Susan

Posted by stedrick
22 Apr 2022, 05:10 PM

My physicians are wondering whether my SCLS is triggering autonomic dysfunction [in paricular, daily hypothermia], or whether the hypothermia is triggering mild flairs. Is there any research on the subject of dysautonomia being a cause rather than a symptom  of SCLS?

I would be most grateful for any information, including fellow patients' experiences. I will be seeing an autonomic neurologist at Massachusetts General Hospital in July, but my physicians are very worried about my temps as low as 90.1 three to four times a day, accompanied by rigor & blue extremities, followed by sweating and bright red extremities & face.

Susan

Insomnia Created by rnuara
Last updated 26 Mar 2022, 03:41 PM

Posted by rnuara
26 Mar 2022, 03:41 PM

As usual, thanks Arturo for your advice.  I especially like your last recommendation.

Posted by aporzeca
25 Mar 2022, 03:21 PM

Robert, first, and as you know, I’m not a physician, and thus the following does not constitute medical advice. Second, FYI, I’ve never had trouble sleeping before, during, or after my IVIG infusions -- but then, I've never taken steroids in conjunction with my infusions.

If I were you, I would discuss the following options with your physician. The first is to reduce or possibly eliminate your intake of steroids, because one of their best-known side effects is that they cause nervousness, anxiety, and insomnia, see, e.g., https://www.hss.edu/conditions_steroid-side-effects-how-to-reduce-corticosteroid-side-effects.asp#:~:text=Insomnia,daily%20dose%20in%20the%20morning and https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7357890/

Therefore, what you are experiencing is unlikely to be a coincidence. Maybe there was a good reason, long ago, for you to take steroids in conjunction with your infusions, but our bodies change over time, so it’s possible that your body doesn’t need those steroids anymore, or not as high a dose of them. Indeed, your having insomnia now may be a signal your body is sending you that it doesn’t want those steroids in your system!

The second option is for you to take a sleeping pill, or something else that will relax you and let you sleep, on those nights after your infusions. Your physician might prescribe you a sleeping medication or tell you to take some Benadryl. He/she probably won’t advise you to take a shot of your favorite alcoholic drink, but that's what some of us do when we have an occasional problem falling asleep!

Good luck!

Posted by rnuara
24 Mar 2022, 08:17 PM

Just wondering if anyone else suffers from insomnia after receiving their IVIG infusions?  I have trouble sleeping each night that they administer the treatment.  Not sure if it just me or not??? They lowered my steroids during the infusion, but that didn't seem to work. If you have trouble sleeping have you found anything to help remedy it/

Does anyone know of a Doctor in the Houston area treating CLSA? Created by Shannoncourt
Last updated 20 Feb 2022, 11:06 AM

Posted by Shannoncourt
20 Feb 2022, 11:06 AM

Aporzeca....

Thank you for your help with the Doctor referrals. I really appreciate it. You have always been a wealth of resources to me through the years. I tried to send you a private message prior to going to the doctor but it apparently did not go through. I see him again this week. I would loove to take you up on your offer to have you forward the articles to my email (Shannoncdowell AT gmail DOT com) . Thank you so much 

 

I have a question for you wondering if you have run in to anyone or heard of anyone else having this issue. I am extremely anemic.  Before leaving Florida I was being worked up to find a reason for this. As this has gone on for years it is nothing for me to hover a H&H around 8-9/28  iron levels low , rdw low etc, in any case when I do go into a SCLS crisis event my h&h will go to a whopping 14/37 which may look normal but is "really high" for me clinically for me. When in reality it isn't high by any standards.

 

thanks for the feedback shannon

trying to get used to Houston but good God oh mighty the traffic is horrible 

Posted by aporzeca
7 Jan 2022, 05:03 PM

I don't recall anyone from Houston having co-authored any published articles on SCLS -- which would be ideal.  However, you can write to Dr. Druey at NIH and ask him if he knows any better and can recommend anyone there.

My generic advice is that you look for a compassionate physician, preferably a senior specialist in internal medicine, immunology, or hematology affiliated with a major university hospital, willing to do his/her homework on this rare disorder (namely, read the literature and follow the advice), and willing to consult with the few SCLS experts available on the topic -- like Dr. Druey.

There are several great hospitals in Houston. For example, the University of Texas Health Science Center is such an academic center, and if you go to their website and look up who's who among the faculty in their Department of Internal Medicine, https://med.uth.edu/internalmedicine/faculty/, you can check out the physicians listed and see who might be a good fit because of what they specialize in (e.g., immune or vascular diseases).

Relevant examples I would consider are https://med.uth.edu/internalmedicine/faculty/rodeo-abrencillo-md/, https://med.uth.edu/internalmedicine/faculty/shervin-assassi-md-ms/, https://med.uth.edu/internalmedicine/faculty/akinsansoye-k-dosekun-bs-mbbs/, or https://med.uth.edu/internalmedicine/faculty/john-d-reveille-md/

At my first encounter, I would hand them a printout of key articles on SCLS found here under Disorder Resources, such as: https://www.jaci-inpractice.org/article/S2213-2198(17)30546-9/fulltext, https://journals.lww.com/ccmjournal/Abstract/2017/07000/The_Clinical_Picture_of_Severe_Systemic.15.aspx and https://www.amjmed.com/action/showPdf?pii=S0002-9343%2817%2930602-2  If you can't access them, let me know via email and I'll email them to you.

Posted by Shannoncourt
6 Jan 2022, 07:10 PM

Hope the new year is treating everyone well. My fiancé is being transferred to the Houston area next month so off we go on another adventure!  Before I set off calling for a new doctor I thought I would ask if anyone knew of someone in that area. 
 

thank you so much!!

shannon

People who have had triple vax and subsequent Covid 19 Infection Created by WazzaACT
Last updated 13 Feb 2022, 05:56 PM

Posted by aporzeca
13 Feb 2022, 05:56 PM

Ariel, if IVIG happens to have the power to abort, shorten, or otherwise minimize an episode of SCLS, well, one would probably want to speed up the infusion process as much as possible.  This is not medical advice, which only your physician can give you, but a logical hypothesis.

My understanding is that the patients in our community all infuse at different speeds allowed by the manufacturer and approved by their physicians.  For example, the manufacturer of Privigen recommends starting out at a speed of 0.5 mg/kg/min (0.005 mL/kg/min) and then increasing the speed up to a maximum of 8 mg/kg/min (0.08 mL/kg/min), see http://cslbehring.vo.llnwd.net/o33/u/central/PI/US/Privigen/EN/Privigen-Prescribing-Information.pdf

In my case, I took the initiative years ago to gradually increase the speed of my infusions, with  my doctor's approval, because I needed to get back to work.  As a result, and for many years now, I have reached the maximum recommended speed, such that I infuse 90 g in less than 3 hours -- the actual time depends on how many bottles are involved (the more, the slower the process) and how available and prompt the nurses are (the slower they are in coming to change the bottles before air bubbles get into the line, the slower the whole process, of course).

Every time I would increase the infusion speed, I would get headaches in the days after, but they subsided with Tylenol, and within 3-4 months my body got used to the faster infusion speeds and I would have no more headaches.  Therefore, I would speed up the process again, which I did until I eventually reached the maximum speed allowed by the manufacturer.

If you are interested in speeding up your regularly scheduled infusion speed, and are willing to go through an adjustment process involving headaches or other unpleasant side effects, please consult your physician and have him/her prescribe the faster infusion target.  That way, should you ever have an episode of SCLS, you will be able to get the IVIG much faster than you do now, and benefit from that.

However, Claude probably holds the world's fastest IVIG infusion record, so you don't have to aim to beat him.  Cutting your infusion process in half the time -- from 6 to 3 hours -- may be a good enough and achievable objective!

Posted by claude53
12 Feb 2022, 06:28 PM

Salut Arielbatt,

Les recommandations des entreprises offrant les médicaments doivent toujours être suivies à la lettre. Parfois, avec un peu d’expérience et en cas d’urgence, on peut adapter les recommandations avec souplesse et bon sens. J’utilise des immunoglobulines depuis 2005, sans avoir eu de problèmes particuliers avec les perfusions. Dans mon cas, je donne ma perfusion mensuelle de 140 g de Privigen (emballé +glucose 5% et 4000 ml) en 2 heures car au-delà de ce temps, une phlébite de la veine perforée commence. J’ai la chance d’être un sportif régulier avec un excellent système cardiovasculaire (sauf pour SCLS). Je pratique tous les jours le vélo ou le ski-alpinisme à un bon niveau. Lors de la réanimation, le temps presse et un taux de perfusion plus rapide que recommandé diminue le risque de choc hypovolémique mortel... le risque de réaction est donc relatif aux avantages. Le 10.02.2020, j’ai commencé la perfusion assez lentement. Après 15 minutes, le débit de perfusion a été augmenté au débit maximal de la ligne veineuse (cathéter court 18 G, débit 105 ml / min) sans aucun problème.

Sinceres salutations. Claude

Posted by Arielbatt
12 Feb 2022, 02:45 PM

Hello everyone, thanks Claude for sharing so quickly.
I have doubts about the rate at which it was infused. He says it was 80Gr in 2 hours. Are those emergency speeds? Or your body always resists that speed without problems. I apply that amount in 6 hours. Is the increase in speed something critical in the emergency that I should alert the doctors?

Ariel.

 

View Full Thread (21 more posts)
Managing IVIG Treatments Created by jenh
Last updated 11 Feb 2022, 06:02 PM

Posted by kmiclon
11 Feb 2022, 06:02 PM

aporzeca - thank you for your input, it is (as usual) helpful and encouraging.  This site continues to be the best resource and truly a comfort when it sometimes feels like I'm navigating blindly thru a snowstorm!

Posted by aporzeca
26 Oct 2021, 03:51 PM

kmiclon,

On your last issue, about having to switch from Gammagard to some other brand of IVIG after the turn of the year, you've got nothing to fear.  There can always be a bad batch once in a million, because after all IVIG is a plasma product and not an artificial, lab-manufactured compound, but the product is generally obtained in a similar manner following stringent quality controls -- no matter the brand.  Over the past dozen years, I have been given Privigen, Octagam, Gammagard and Gamunex-C, and I've had no adjustment problems at all.

The reason why different health systems, hospitals, and clinics use different brands is because they shop around and make their own deals with different manufacturers.  And I think the most common reason why they sometimes "run out" of a particular brand is probably because they are wanting to impress upon their current provider that they are willing to switch brands if they can get better pricing.

Posted by kmiclon
24 Oct 2021, 11:19 PM

I started IVIG about 2 years ago at full dosage of 2g/kg over 2 days at a local infusion center, and had migraines and felt 'hungover' for about a week after.  I did realize about 2 weeks in, though, that my constant leak of 10 years was completely gone, so I contacted my Mayo doctor and asked about stopping, but he preferred to be proactive instead of reactive, and we compromised by stretching that to 8 weeks.  After 2 or 3 more treatments with a week to 10 days of recovery each, we adjusted to 1g/kg every 4 weeks. I've been on that schedule for almost a year now and am still not leaking and have not had any kind of episode since beginning. I do so appreciate the willingness of my doctors to listen and respect my opinion -

For prep, the week of my Friday treatment I increase fluid intake, (attempt to) eat cleanly, avoid alcohol, and get extra rest. I'm lucky to have my treatments at home, my nurse shows up about 9 and is gone by 1. I am a network engineer working from home, and able to work thru my treatment. In the beginning, I was missing at least 1 day/week after treatment with headaches & 'hangover', but the last few months I have been very close to not even noticing I've had a treatment. My husband has concerns about this being something we have to do/schedule/figure out for a lifetime, but I have come to look at it as luxury to slow down and take care of myself, listen to my body, eat and rest as I feel the need - and I am a strong believer that IVIG is worth it until something better comes along.

I am very lucky to have good veins, but I do have concerns about them becoming difficult with so much use.  I'm not interested in a port right now. I have been talking a bit with my nurse about the subcutaneous IG, maybe at a later time.

As far as travel, we will plan around my treatment. I, like Hans, am petrified of having an episode in another country, but we will bring Mayo info and contact wherever/whenever we go.

As a sort of side note, I have been getting Gammagard, but my insurance will not pay for it after January 1, and I have heard that the different brands can have very different reactions, so am a bit afraid that I may have to start figuring out how to cope post-treatment all over. I am going to look here on Rareshare for other patients' reactions to them. This site has been a blessing, you will find so much help here - good luck to you :-) 

View Full Thread (6 more posts)
Getting the Booster when the Vaccine Triggered an Attack Created by jenh
Last updated 28 Jan 2022, 12:10 PM

Posted by krogers
28 Jan 2022, 12:10 PM

Hi Kevin,

I was in effect self diagnosed and was in your position several years ago. The key to confirming your diagnosis is the blood test result when you were ill. If you see Heamoglobin concentration during your illness then this conforms the condition. I think its the same for albumin levels. This shows fluid is leaking out of your capillary blood vessels so the blood volume decreases hence the concentration of the above goes up. It will return to normal when you get better. All the information you need is on the information part of this site but you may have to push your doctors to look into this as I did. Referral to Dr Drury at the NIH is then your next step - he is the world expert on this condition. Cheers, Another Kevin

Posted by klafata1970
27 Jan 2022, 07:09 PM

Good afternoon... I've just gotten home from a hospital discharge brought on by what seems to be very similar symptoms 48 hours after my Covid booster (Pfizer).  Doctors have yet to come up with any kind of diagnosis for multiple hospital visits in the same cirumstances (2nd covid shot, flu vaccine in 2020).  I was finally fed up with not knowing, and after many many google searched, I ended up here. So far everyone's experience is the most similar to any other possible condition I've seen.  Not sure I can add anything of value to this thread at the moment, but wanted to post my first message after joining the community.

- (Another) Kevin

Posted by DudeSCLS
16 Dec 2021, 01:56 PM

Hi,

I think I echo a few voices in this group when I say that you're not alone. The fear, anxiety, ptsd, and/or panic attacks. It's hard to be comfortable with SCLS during flu season, significantly more through a pandemic. It's hard to have complete confidence in medicine. Especially when Dr. Druey says "...nothing is full proof...". But I have to echo the late George Micheal as well, "you gotta have faith" in IVIG (and a higher power if your into that). There's a reason I'm on the highest IVIG dose possible. Because I can, I know like a miracle it can and has saved those with SCLS, and as long as I can I will receive IVIG until the doc comes up with another preventive treatment or cure. I understand your hesitation with getting the booster. It's a lot to dissect. My second SCLS attack was because of the flu. I got diagnosed then. I even got my flu vaccine beforehand. My point goes back to Dr. Druey's words. Is it possible that you could have another attack ever again once you're on IVIG? Sure. But it's probably not going to happen and if it does IVIG will probably save your life without the need for life support. Full disclosure, I've received both moderna vaccines and booster with no SCLS attacks and I'm receiving 2g/kg IVIG every 4 weeks.

Getting diagnosed with SCLS stinks, but it beats not having a diagnosis at all and having no IVIG support. Don't hesitate to reach out if you need to talk. 

- Kevin
 

View Full Thread (2 more posts)
Community News Articles
Default article

Hospitals, Doctors, Medical Teams: Navigating Barriers of Rare Diseases (Patient Navigation)

Publication date: 12 Sep 2016

Community: Systemic Capillary Leak Syndrome

Podcast

Arturo Porzecanski, a rare disease patient and advocate, gives us some tips on navigating decisions involved in choosing hospitals, doctors, and medical teams.
Featuring Arturo Porzecanski (American University). (Music credit:www.bensound.com)


Community Resources
Title Description Date Link
The Consequences of the Covid-19 Pandemic on SCLS Patients

The Consequences of the Covid-19 Pandemic on SCLS Patients

Abstract: The authors report on the fate of 30 known SCLS patients from Europe through mid-July 2021: 90% (27) of them were receiving IVIG on a regular basis, and two-thirds (20) of them were vaccinated. Five of the ten who were unvaccinated patients experienced an episode of SCLS and 4 of them died as a result, even though none had evidence of Covid-19 pneumonia. Covid vaccination was uneventful in 18 out of the 20 patients, including 2 who were not receiving IVIG. Two patients treated with IVIG had a relapse after a second dose of mRNA vaccine, with a favorable outcome in both cases. In addition, five patients were newly diagnosed with SCLS, none of whom were receiving IVIG: 4 of them were unvaccinated and had an episode of SCLS after contracting Covid, and the 5th one after receiving a first Covid vaccination. One of the unvaccinated four died, while the rest survived. In sum, the Covid pandemic has had serious consequences in patients with SCLS. Covid infections are associated with a high risk of SCLS episodes, and all Covid vaccines can trigger episodes. High-dose IVIG remains the only effective preventive treatment and should not be stopped during the pandemic. The risk/benefit ratio favors Covid vaccination in SCLS patients receiving IVIG.

01/07/2022
Chronic SCLS treatment with IVIG: Case & literature

Chronic systemic capillary leak syndrome treatment with intravenous immune globulin: Case report and review of the literature 

Abstract: A rarely described chronic form of SCLS (cSCLS) presents as refractory edema, with pleural and/or pericardial effusions and hypoalbuminemia. These entities are differentiated by massive and periodic episodes of capillary leak, which can result in shock in SCLS, and chronic refractory edema in cSCLS. The etiologies of these disorders are poorly understood, but both acute and chronic forms often present with an associated monoclonal gammopathy. Flares of the SCLS have been reduced by treatment with intravenous immune globulin (IVIG). Only six cases of cSCLS have been reported, and previous treatments have included steroids, terbutaline, and theophylline. Based upon the reported responses of SCLS to IVIG, we present the case of a 54-year-old man with cSCLS where ongoing treatment with IVIG resulted in a marked and sustained improvement in the signs and symptoms of the capillary leak syndrome.

01/07/2022
Handling Shock in SCLS: Less Is More

Handling shock in idiopathic systemic capillary leak syndrome (Clarkson’s disease): less is more 

Abstract: SCLS presents with recurrent potentially life-threatening episodes of hypovolemic shock associated with severe hemoconcentration and hypoproteinemia. Here the authors summarize 40 years’ experience in treating shock in Italian SCLS patients to derive a therapeutic algorithm. Records from 12 patients were informative for treatment modalities and outcome of 66 episodes of shock. Episodes are divided in 3 phases and treatment recommendations are the following: (1) prodromal symptoms-signs (growing malaise, oligo-anuria, orthostatic
dizziness) last 6-12 hours and patients should maintain rigorous bed rest. (2) The acute shock phase lasts 24-36 hours; patients should be admitted to ICU, placed on restrictive infusion of fluids favoring cautious boluses of high-molecular-weight plasma expanders when SAP < 70 mmHg; and monitored for cerebral/cardiac perfusion, myocardial edema and signs of compartment syndrome. (3) The post-acute (recovery) phase may last from 48 hours to 1 week; monitor for cardiac overload to prevent cardiac failure; in case of persistent renal failure, hemodialysis may be necessary; consider albumin infusion. Complications listed by frequency in our patients were acute renal failure, compartment syndrome and neuropathy, rhabdomyolysis, myocardial edema, pericardial-pleural-abdominal effusion, cerebral involvement, acute pulmonary edema and deep vein thrombosis.

01/07/2022
A natural mouse model reveals genetic determinants of SCLS

A natural mouse model reveals genetic determinants of systemic capillary leak syndrome

Abstract: SCLS is a disorder of unknown etiology characterized by recurrent episodes of vascular leakage of proteins and fluids into peripheral tissues, resulting in whole-body edema and hypotensive shock. The pathologic mechanisms and genetic basis for SCLS remain elusive. Previous histological studies of skin and muscle of SCLS patients have failed to uncover gross abnormalities within the microvasculature that could account for this phenotype. Here the authors identified an inbred mouse strain, SJL, which recapitulates cardinal features of SCLS, including susceptibility to histamine- and infection-triggered vascular leak. They named this trait “Histamine hypersensitivity” (Hhs/Hhs) and mapped it to Chromosome 6. Hhs is syntenic to the genomic locus most strongly associated with SCLS in humans (3p25.3), revealing that the predisposition to develop vascular hyperpermeability has a strong genetic component conserved between humans and mice and providing a naturally occurring animal model for SCLS. Genetic analysis of Hhs may reveal orthologous candidate genes that contribute not only to SCLS, but also to normal and dysregulated mechanisms underlying vascular barrier function more generally. Future studies including assessment of expression and sequence of top Hhs candidate genes in SCLS patients and mice and their role in endothelial responses to inflammation will be essential to determine their contribution.

01/07/2022
Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated SCLS

Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated SCLS

Abstract: We conducted a cohort analysis of all patients included in the European Clarkson disease registry between January 1997 and March 2016. From diagnosis to last follow-up, studied outcomes (e.g., the frequency and severity of attacks, death, and evolution toward multiple myeloma) and the type of preventive treatments administered were monitored every 6 months. Sixty-nine patients (M/F sex ratio 1:1; mean ± SD age at disease onset 52 ± 12 years) were included in the study. All patients had monoclonal gammopathy of immunoglobulin G type, with kappa light chains in 47 (68%).Twenty-four patients (35%) died after 3.3 (0.9-8) years. Fifty-seven (86%) patients received at least one preventive treatment, including intravenous immunoglobulins (IVIg) n = 48 (73.8%), theophylline n = 22 (33.8%), terbutaline n = 22 (33.8%), and thalidomide n = 5 (7.7%). In the 65 patients with follow-up, 5- and 10-year survival rates were 78% (n = 35) and 69% (n = 17), respectively. Preventive treatment with IVIg and terbutaline were the only factors significantly associated with survival in multivariate analysis. Neither the use of thalidomide nor theophylline was associated with improved survival. Five- and 10-year survival rates in patients treated with IVIg were 91% and 77%, respectively, compared to 47% and 37% in patients not treated with IVIg. Patients treated with IVIg were more likely to be free of recurrence, severe recurrence, and alive at the end of follow-up. Furthermore, all but one patient who did not experience a severe relapse were treated with IVIg. Since preventive treatment with IVIg was the strongest factor associated with survival, the use of IVIg is suggested as the first line in prevention therapy.

01/07/2022
Whole Exome Sequencing of SCLS Patients

Whole Exome Sequencing of Adult and Pediatric Cohorts of the Rare Vascular Disorder Systemic Capillary Leak Syndrome

Abstract: The extent to which genetic abnormalities contribute to SCLS is unknown. The authors identified pediatric and adult cohorts with characteristic clinical courses and sought to identify a possible genetic contribution to SCLS through the application of Whole Exome Sequencing (WES). On the basis of 9 adult and 8 pediatric SCLS patients and available unaffected first-degree relatives, they did not identify a uniform germline exomic genetic etiology for SCLS. However, WES did identify several candidate genes for future research.

11/04/2018
Idiopathic SCLS (Clarkson syndrome) in childhood

Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review

Abstract: The authors performed a systematic review of the literature on Clarkson syndrome in subjects less than 18 years of age, and identified 24 reports, published since 1989, providing data on 32 otherwise healthy subjects, who experienced 67 well-documented episodes of SCLS. The condition affected more frequently girls (21, 66%) than boys, presented throughout childhood, and was preceded by a mostly viral illness in 75% of cases. The presence of a monoclonal gammopathy (MGUS) was never reported. Uncompensated circulatory shock, muscle compartment syndrome, acute kidney injury, pulmonary edema, and either pleural or pericardial effusion were, in decreasing order of frequency, the most common complications. Four patients died. In sum, SCLS develops not only in adulthood but also in childhood, but of potential significance is that in this age group the condition is not linked to an MGUS, and thus it could be that it does not play a pivotal pathogenic role.

11/04/2018
Clinical Presentation, Management, and Prognostic Factors of SCLS

Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review

Abstract: A total of 133 case reports (161 patients) and 5 case series (102 patients) of idiopathic SCLS were included in a survey of articles published through end-2016. The findings include that patients had hypotension (81.4%), edema (64.6%), and previous flu-like illness (34.2%). They were often misdiagnosed as having hypovolemic shock, septic shock, polycythemia vera, or angioedema. Thirty-seven patients died (23%) mainly because of complications from SCLS (78.4%). There were significant differences in the survival rates between patients who were treated with prophylactic b2 agonists, methylxanthines, and intravenous immunoglobulins and those who were not. The estimated 1-, 5-, and 10-year survival rate of patients treated with intravenous immunoglobulins was 100%, 94%, and 94%, respectively. The results of this review suggest that prophylactic use of intravenous immunoglobulins is the most effective treatment in reducing the mortality rate of SCLS patients.

11/04/2018
The Clinical Picture of Severe SCLS Episodes Requiring ICU Admission

The Clinical Picture of Severe SCLS Episodes Requiring ICU Admission

Abstract: SCLS is a very rare cause of recurrent hypovolemic shock. Few data are available on its clinical manifestations, laboratory findings, and outcomes of those patients requiring ICU admission.  This study was undertaken to describe the clinical pictures and ICU management of severe SCLS episodes.  This multicenter retrospective analysis concerned patients entered in the European Clarkson's disease (EurêClark) Registry and admitted to ICUs between May 1992 and February 2016.  Fifty-nine attacks occurring in 37 patients (male-to-female sex ratio, 1.05; mean ± SD age, 51 ± 11.4 yr) were included.  Among 34 patients (91.9%) with monoclonal immunoglobulin G gammopathy, 20 (58.8%) had kappa light chains.  ICU-admission hemoglobin and proteinemia were respectively median (interquartile range) 20.2 g/dL (17.9-22 g/dL) and 50 g/L (36.5-58.5 g/L).  IVIG was infused during 15 episodes (25.4%).  A compartment syndrome developed during 12 episodes (20.3%).  Eleven (18.6%) in-ICU deaths occurred. Bivariable analyses (the 37 patients' last episodes) retained Sequential Organ-Failure Assessment score greater than 10 (odds ratio, 12.9 [95% CI, 1.2-140]; p = 0.04) and cumulated fluid-therapy volume greater than 10.7 L (odds ratio, 16.8 [1.6-180]; p = 0.02) as independent predictors of hospital mortality.  In conclusion, high-volume fluid therapy was independently associated with poorer outcomes.  IVIG use was not associated with improved survival; hence, its use in an ICU setting should be considered prudently and needs further evaluation in future studies.

07/05/2017
Capillary leak syndrome: etiologies, pathophysiology, and management

Capillary leak syndrome: etiologies, pathophysiology, and management

Abstract: In various human diseases, an increase in capillary permeability to proteins leads to the loss of protein-rich fluid from the intravascular to the interstitial space. Although sepsis is the disease most commonly associated with this phenomenon, many other diseases can lead to a “sepsis-like” syndrome with manifestations of  diffuse pitting edema, exudative serous cavity effusions, noncardiogenic pulmonary edema, hypotension, and, in some cases, hypovolemic shock with multiple-organ failure. The term capillary leak syndrome has been used to describe this constellation of disease manifestations associated with an increased capillary permeability to proteins. Diseases other than sepsis that can result in capillary leak syndrome include the idiopathic systemic capillary leak syndrome or Clarkson’s disease, engraftment syndrome, differentiation syndrome, the ovarian  hyperstimulation syndrome, hemophagocytic lymphohistiocytosis, viral hemorrhagic fevers, autoimmune diseases, snakebite envenomation, and ricin poisoning. Drugs including some interleukins, some monoclonal antibodies, and gemcitabine can also cause capillary leak syndrome. Acute kidney injury is commonly seen in all of these diseases. In addition to hypotension, cytokines are likely to be important in the pathophysiology of acute kidney injury in capillary leak syndrome. Fluid management is a critical part of the treatment of capillary leak syndrome;  hypovolemia and hypotension can cause organ injury, whereas capillary leakage of administered fluid can worsen organ edema leading to progressive organ injury. The purpose of this article is to discuss the diseases other than sepsis that produce capillary leak and review their collective pathophysiology and treatment.

03/24/2017
Idiopathic systemic capillary leak syndrome (Clarkson disease)

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Abstract: The enigmatic systemic capillary leak syndrome (SCLS) named for Dr Clarkson is characterized by transient and severe but reversible hemoconcentration and hypoalbuminemia caused by leakage of fluids and macromolecules into tissues. Although less than 500 cases of SCLS have been reported in the literature since 1960, the condition is probably underdiagnosed because of a lack of awareness and a high mortality without treatment. Treatment of acute SCLS remains primarily supportive. Prophylaxis with IVIG appears promising, but this therapy is nonspecific and expensive. Mechanistic understanding of SCLS is in its infancy. As a result, clinicians today cannot predict when or how badly SCLS will flare; targeted therapies do not yet exist, and prolonged remission or cure remains elusive. Our working hypothesis invokes exaggerated microvascular endothelial responses to surges of otherwise routinely encountered inflammatory mediators. This emerging disease model lends itself to innovative patient-centered translational research in the ways highlighted above. It is our hope that detailed and personalized investigation of intraendothelial responses among individual patients with SCLS might illuminate novel genetic and molecular control mechanisms. In turn, such advances could deliver the diagnostic, prognostic, and therapeutic tools sorely needed to combat this devastating disease.

03/23/2017
Sharing the Pain [of living with SCLS]

Sharing the Pain [of living with SCLS]

This article from The Washington Post newspaper tells the story of how this SCLS virtual community was created, the story of its founder and, more generally, of this fantastic RareShare site.

 

03/23/2017
The Mayo Clinic Experience with SCLS

Idiopathic Systemic Capillary Leak Syndrome (Clarkson's Disease): The Mayo Clinic Experience

Abstract: Of the 34 patients whose records were reviewed, 25 fulfilled all diagnostic criteria for SCLS. The median age at diagnosis of SCLS was 44 years. Median follow-up of surviving patients was 4.9 years, and median time to diagnosis from symptom onset was 1.1 years (interquartile range, 0.5-4.1 years). Flulike illness or myalgia was reported by 14 patients (56%) at onset of an acute attack of SCLS, and rhabdomyolysis developed in 9 patients (36%). Patients with a greater decrease in albumin level had a higher likelihood of developing rhabdomyolysis (P=.03). Monoclonal gammopathy, predominantly of the IgG-kappa type, was found in 19 patients (76%). The progression rate to multiple myeloma was 0.7% per person-year of follow-up. The overall response rate to the different therapies was 76%, and 24% of patients sustained durable (>2 years) complete remission. The estimated 5-year overall survival rate was 76% (95% confidence interval, 59%-97%). In conclusion, SCLS, a rare disease that occurs in those of middle age, is usually diagnosed after a considerable delay from onset of symptoms. The degree of albumin decrement during an attack correlates with development of rhabdomyolysis. A reduction in the frequency and/or the severity of attacks was seen in nearly three-fourths of patients who were offered empiric therapies. The rate of progression to multiple myeloma appears to be comparable to that of monoclonal gammopathy of undetermined significance.

03/23/2017
Genome-Wide SNP Analysis of SCLS

Genome-Wide SNP Analysis of SCLS.

Abstract: Polymorphisms in genes whose functional annotations suggest involvement in cell junctions and signaling, cell adhesion, and cytoskeletal organization, correlate with our previous mechanistic studies of SCLS sera. Such annotations provide a framework for future allelic discrimination strategies to validate top-ranked SNPs discovered here, as well as novel SNPs unique to the SCLS cohort detected by exome capture sequencing. Although the findings must be corroborated in a larger cohort, they provide a springboard for discovery of underlying pathophysiological mechanisms, biomarkers, and avenues for therapy.

03/23/2017
IVIG in SCLS: A Case Report and Review of Literature

IVIG in SCLS: Report and Review of Literature.

Abstract: In recent years, IVIG has become a common first-line prophylactic therapy in most patients with benefits at the dose of 2 gr/kg once a month. Here the authors report the case of a 49-year-old male patient in Italy -- he is a member of this community -- with SCLS treated successfully with a lower dose of IVIG (1 gr/kg monthly) in the maintenance phase. He presented no acute episodes in a follow-up period of 28 months. The authors describe prophylactic treatments for SCLS in the literature and compare their patient to another 18 who received IVIG in follow-up.

03/23/2017
Mechanistic Classification of SCLS

Mechanistic Classification of SCLS.

Abstract: The authors analyzed circulating mediators of vascular permeability and proinflammatory cytokines in acute episodic sera from 14 patients with SCLS, and sera from 37 healthy control subjects. They monitored barrier function of human microvascular endothelial cells (HMVEC) after treatment with SCLS sera using transendothelial electrical resistance assays. Consistent with their previous study, the permeability factor vascular endothelial growth factor (VEGF) was increased in sera from acutely ill subjects with SCLS. An analysis of samples from one SCLS patient who has not responded to any preventive therapies (and who is a member of this Community), suggests that SCLS may have clinically varying forms, and that within the group of patients with SCLS, different cytokines may mediate the capillary leak. Therefore, quantitative molecular and humanized cell-based assays for humoral mediators of permeability should improve diagnostic specificity for SCLS and enable clinicians to screen for effective therapies.

03/23/2017
High-Dose IVIG Therapy for SCLS

High-Dose IVIG Therapy for SCLS.

Abstract: We evaluated IVIG prophylactic therapy in a cohort of 29 patients with Systemic Capillary Leak Syndrome in a longitudinal follow up study. All patients received treatments at the discretion of their primary providers and retrospectively via questionnaire recorded symptoms beginning with their first documented episode of the SCLS until May 31, 2014. Twenty-two out of 29 patients responded to the questionnaire, and 18 out of the 22 respondents received monthly prophylaxis with IVIG during the study period for a median interval of 32 months. The median annual attack frequency was 2.6/patient prior to IVIG therapy and 0/patient following initiation of IVIG prophylaxis (P = 0.001). 15 out of 18 subjects with a history of one or more acute SCLS episodes experienced no further symptoms while on IVIG therapy. In conclusion, IVIG prophylaxis is associated with a dramatic reduction in the occurrence of SCLS attacks in most patients, with minimal side effects.

03/23/2017
SCLS in Children

Idiopathic Systemic Capillary Leak Syndrome in Children

Abstract: Adult subjects with systemic capillary leak syndrome (SCLS) present with acute and recurrent episodes of vascular leak manifesting as severe hypotension, hypoalbuminemia, hemoconcentration, and generalized edema. We studied clinical disease characteristics, serum cytokine profiles, and treatment modalities in a cohort of children with documented SCLS. Six children with SCLS were recruited from the United States, Australia, Canada, and Italy. Serum cytokines from SCLS subjects and a group of 10 healthy children were analyzed. Children with SCLS (aged 5-11 years old) presented with at least 1 acute, severe episode of hypotension, hypoalbuminemia, and hemoconcentration in the absence of underlying causes for these abnormalities. In contrast to what is observed in adult SCLS, identifiable infectious triggers precipitated most episodes in these children, and none of them had a monoclonal gammopathy. We found elevated levels of chemokine (C-C motif) ligand 2 (CCL2), interleukin-8, and tumor necrosis factor α in baseline SCLS sera compared with the control group. All patients are alive and well on prophylactic therapy, with 4 patients receiving intravenous or subcutaneous immunoglobulins at regular intervals. The clinical manifestations of pediatric and adult SCLS are similar, with the notable exceptions of frequent association with infections and the lack of monoclonal gammopathy. Prophylactic medication, including high dose immunoglobulins or theophylline plus verapamil, appears to be safe and efficacious therapy for SCLS in children.

03/23/2017
Systemic capillary leak syndrome: recognition prevents morbidity and mortality

Systemic capillary leak syndrome: recognition prevents morbidity and mortality.

Abstract: The authors report on a case of SCLS in Australia involving a 61-year-old male who was properly diagnosed after his third episode, to increase awareness of the condition and to highlight the benefits of prophylactic intravenous immunoglobulin (IVIG) in this condition. The diagnosis was made by exclusion and clinically by a classic triad of hypotension, hypoalbuminaemia and haemoconcentration. There have been recent advances in understanding the pathophysiological basis for SCLS and in effective prophylaxis, and the authors and patient benefitted from said advances.

03/23/2017
Laboratory Evidence of SCLS and of the Effectiveness of IVIG

Vascular Endothelial Hyperpermeability Induces The Clinical Symptoms of Clarkson Disease (The Systemic Capillary Leak Syndrome)

Abstract: The authors report clinical and molecular findings on 23 subjects, the largest SCLS case series to date. Application of episodic SCLS sera, but neither the purified immunoglobulin fraction nor sera obtained from subjects during remission, to human microvascular endothelial cells caused vascular endothelial cadherin (VE-cadherin) internalization, disruption of inter-endothelial junctions, actin stress fiber formation, and increased permeability in complementary functional assays without inducing endothelial apoptosis. Intravenous immunoglobulin (IVIG), one promising therapy for SCLS, mitigated the permeability effects of episodic sera directly. Consistent with the presence of endogenous, non-immunoglobulin, circulating permeability factor(s) constrained to SCLS episodes, we found that two such proteins, vascular endothelial growth factor (VEGF) and angiopoietin 2 (Ang2), were elevated in episodic SCLS sera but not in remission sera. Antibody-based inhibition of Ang2 counteracted permeability induced by episodic SCLS sera. Comparable experiments with anti-VEGF antibody (bevacizumab) yielded less interpretable results, likely due to endothelial toxicity of VEGF withdrawal. Our results support a model of SCLS pathogenesis in which non-immunoglobulin humoral factors such as VEGF and Ang2 contribute to transient endothelial contraction, suggesting a molecular mechanism for this highly lethal disorder.

03/23/2017
Successful Treatment of SCLS with IVIG

Successful Treatment of Systemic Capillary Leak Syndrome with Intravenous Immunoglobulins.

Abstract: The authors report on a 48-year-old woman in Spain who had her 1st episode of SCLS in 1997 and was initially put on a regimen of terbutaline and aminophylline, but went on to endure 20 additional episodes in the subsequent 3 years. She was then treated with melphalan-prednisone for a year and the frequency and intensity of her episodes diminished and even disappeared. In 2005, however, the episodes returned and in 2008 she was finally put on a regimen of IVIG (2 g/kg) every 6 weeks. She has had no more episodes since then.

03/23/2017
Comment on SCLS

Comment on The Systemic Capillary Leak Syndrome.

Abstract: The authors report on 2 additional patients from the United States with SCLS in whom prophylaxis with terbutaline and theophylline failed, but who had no further episodes after the initiation of IVIG therapy. There are additional published reports of successful prophylaxis with IVIG cited, and the authors are also aware of yet another case. Given the present state of knowledge and despite the high cost, the authors strongly believe that IVIG is the optimal prophylaxis and should be the initial choice to prevent attacks in patients with SCLS.

03/23/2017
IVIG: A Promising Approach to SCLS

High-dose intravenous immunoglobulins: A promising therapeutic approach for idiopathic systemic capillary leak syndrome.

Abstract: The article reports the case of a 40-year-old woman with chronic SCLS treated in Berne, Switzerland, with high-dose intravenous immunoglobulins (IVIG) after a prophylactic therapy with theophylline and terbutaline (T&T) was poorly tolerated and failed to decrease the frequency and severity of the attacks. During the 5 years she was on T&T the patient suffered from about 20 similar episodes of mild to moderate shock, often requiring hospital re-admission and supportive therapy. So far, 10 months of prophylactic therapy with IVIG (2gr/kg/month) have resulted in an impressive reduction of intensity and frequency of attacks, confirming the finding of other case studies.

03/23/2017
Lessons from 28 European Patients with SCLS

The Systemic Capillary Leak Syndrome: A Case Series of 28 Patients From a European Registry.

Abstract: The article describes the clinical characteristics, laboratory findings, treatments, and outcomes of patients with SCLS who were not previously reported in the literature. These European patients with SCLS were treated and monitored from the start of 1997 until end-July 2010. Survival rates were 89% at 1 year and 73% at 5 years; instances of death were directly related to SCLS attacks in 6 cases (75% of total). Treatments of various kinds increased the chances of survival: Five years after diagnosis, survival rates were 85% in 23 patients who had received a treatment and just 20% in 5 patients who had not. The authors provide additional evidence that a prophylactic treatment with IVIG tends to reduce the frequency and severity of attacks, and may improve the survival of patients with SCLS.

03/23/2017
Mayo Clinic write-up on SCLS

The Mayo Clinic's summary of the diagnosis and treatment of SCLS.

During an episode of systemic capillary leak syndrome, fluids are administered intravenously to maintain the patient's blood pressure and to prevent damage to vital organs such as the kidneys, heart and brain. The amount of fluid must be carefully controlled. An attempt to normalize blood pressure through aggressive fluid administration can cause destructive swelling of the body's extremities and overload the kidneys and lungs when the body needs to eliminate the excess fluids after the episode passes.

Glucocorticoids (steroids) are often injected during an acute capillary leak syndrome attack to reduce or stop the capillary leak. This is sometimes successful. Fluid pressure in muscles may be monitored. Emergency surgery may be needed to relieve pressure and minimize damage to muscles and nerves in the arms and legs.

Once the capillary walls stop leaking and fluids start to be reabsorbed, patients are usually given diuretics to speed up elimination of the fluids before they accumulate in the lungs and other vital organs, which can be a fatal complication.

Patients who avoid organ and limb damage in a capillary leak syndrome episode tend to recover their health after several days, once the capillary walls return to normal and the accumulated fluid is expelled from the body through urination.

Although no cure has been found for systemic capillary leak syndrome, the frequency and/or severity of episodes is often reduced by having patients take certain asthma medications: theophylline and terbutaline. Patients also may benefit from intravenous treatment with immunoglobulin or by taking thalidomide.

Patients may also be prescribed corticosteroid pills such as prednisone to be taken at the first sign of symptoms of another capillary leak.

03/23/2017
IVIG as Treatment for SCLS

Immunoglobulins for Treatment of Systemic Capillary Leak Syndrome

Abstract: A 43-year-old white woman in France diagnosed with SCLS was put on the recommended combination of Theophylline plus Terbutaline, but she nevertheless had 10 episodes of severe capillary leak during 2001-mid-2007, necessitating intensive care unit admission for her last 3 episodes. She was then put on IVIG administered every 6 weeks, and this yielded a dramatic improvement such that she has had no more episodes and has returned to her normal lifestyle.

03/23/2017
IVIG as Treatment for SCLS

High-Dose Intravenous Immunoglobulins Dramatically Reverse Systemic Capillary Leak Syndrome.

Abstract: The objective of this study was to report the dramatic improvement of patients with systemic capillary leak syndrome obtained with high-dose intravenous immunoglobulins. Systemic capillary leak syndrome is a rare and life-threatening disorder characterized by hypotension that can lead to shock, weight gain, hypoalbuminemia, and elevated hematocrit secondary to unexplained episodic capillary fluid extravasation into the interstitial space. Because its cause is unknown, systemic capillary leak syndrome treatment has remained largely supportive. Intravenous immunoglobulins administration to a patient with refractory systemic capillary leak syndrome yielded dramatic improvement. The patient is still alive 11 yrs after systemic capillary leak syndrome diagnosis and receives intravenous immunoglobulins monthly. Later, based on that result, intravenous immunoglobulins were successfully given to two other patients during the acute phase of systemic capillary leak syndrome. Both are still alive 8 and 1.5 yrs after receiving intravenous immunoglobulins at the onset of each flare. In conclusion, intravenous immunoglobulins were effective against systemic capillary leak syndrome symptoms in three patients, but their exact mechanism remains unknown. Their immunomodulatory effect merits further investigation.

03/23/2017
The Systemic Capillary Leak Syndrome

Narrative review: the systemic capillary leak syndrome

Abstract: The systemic capillary leak syndrome (SCLS) is a rare disease of reversible plasma extravasation and vascular collapse accompanied by hemoconcentration and hypoalbuminemia. Its cause is unknown, although it is believed to be a manifestation of transient endothelial dysfunction due to endothelial contraction, apoptosis, injury, or a combination of these. Fewer than 150 cases of SCLS have been reported, but the condition is probably underrecognized because of its nonspecific symptoms and signs and high mortality rate. Patients experience shock and massive edema, often after a nonspecific prodrome of weakness, fatigue, and myalgias, and are at risk for ischemia-induced organ failure, rhabdomyolysis and muscle compartment syndromes, and venous thromboembolism. Shock and edema reverse almost as quickly as they begin, at which time patients are at risk for death from flash pulmonary edema during rapid fluid remobilization. Diagnosis is made clinically and by exclusion of other diseases that cause similar symptoms and signs, most notably sepsis, anaphylaxis, and angioedema. Acute episodes are treated with vasopressor therapy and judicious fluid replacement, possibly with colloid solutions for their osmotic effects, to prevent the sequelae of underperfusion. Prognosis is uncertain, but patients who survive an initial severe SCLS episode are estimated to have a 10-year survival rate greater than 70%. Much remains to be learned about SCLS, and clinicians should consider the diagnosis in patients with unexplained edema, increased hematocrit, and hypotension.

03/23/2017

Clinical Trials


Cords registry

CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access.

Enrolling is easy.

  1. Complete the screening form.
  2. Review the informed consent.
  3. Answer the permission and data sharing questions.

After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.

Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.

Visit sanfordresearch.org/CoRDS to enroll.

Community Leaders

aporzeca

I had my first episode of what turned out to be SCLS in November 2005, and was very lucky to have survived it (though with permanent disabilities in arms and legs, and thus in hands and feet) and to have been diagnosed correctly within days.

I went on to have 2 other life- and limb-threatening episodes in April 2007 and March 2009, requiring 2+ weeks of Intensive Care hospitalization to keep my organs alive and emergency fasciotomies to preserve the muscles and nerves I still have in my extremities.

I also had 7 episodes of lesser severity (Dec. 2007, June 2008, June 2009, July 2009, September 2009, and two in November 2009), because I realized I was having them early on, which allowed me to get a massive dose of steroids (Prednisone pills and/or injections of Solu-Medrol and Albumin) that effectively stopped the capillary leak phase of SCLS.

Given the increased frequency of my episodes of SCLS, despite having taken the recommended doses of the traditional medications (e.g., Theophylline, Terbutaline and Singulair), I was given my first infusion of IVIG in November 2009 and have had monthly infusions since then with no adverse side effects whatsoever. So far, so very good: for over a decade I had no more episodes of SCLS, though I did have one in February 2020, after coming down with Influenza A days before I was due for my next IVIG infusion, which I hope will prove to be the exception to the rule.

While I was among the first SCLS patients in the United States to benefit from an IVIG therapy, most other patients who had previously been getting this medication in Europe, and virtually all patients around the world who have since received IVIG, have stopped having episodes of SCLS.

Our stories are now told in a number of case studies published in various medical journals, and there is also a scientific article showing the efficacy of IVIG in countering SCLS in laboratory conditions based on our blood samples before and after receiving IVIG, as well as several articles with the results of surveys of SCLS patients who have been on IVIG.  The evidence that IVIG is the best and almost always successful therapy for the prevention of episodes of SCLS is now overwhelming.

My personal email address is aporzeca@american.edu

 

Expert Questions

Ask a question

genecridge Message
9 Sep 2014, 11:57 PM

I recently lost my job on the basis of a letter sent to my company from my specialist. He was asked to explain SCLS to my employer in order to put them at ease with my condition. My work involves long-haul travel with visits to Third World countries involved, on occasions. I recently missed 2 treatments of the IVIG and this resulted in my having 2 episodes, but I am now back on IVIG, and it should be reasonable to assume that now all will be as well as before, given the evidence that IVIG seems to have stopped episodes of SCLS in most if not all cases.

Answer

I am terribly sorry to hear about your losing your job!

In many countries around the world, employers can fire their workers at any time, for any reason, unless they are specifically protected by clauses in a contract or by legislation. After all, attendance is a basic job requirement for most positions.

However, there may be laws that might protect you from being fired for taking sick leave. Now that your employer has fired you, you should speak to a labor lawyer right away, to ensure that your rights were not violated.

An experienced employment lawyer can assess your situation and help you decide how to proceed. And since you have been fired even though relevant medical evidence appears to have been missed by your specialist, and even though you could have been given another chance, a lawyer can help you negotiate a fair severance -- or file a legal claim against your employer, if that's the best strategy in your situation.

I hope you will stick to a regular course of IVIG therapy from now on, and that it will work as well for you as it has for virtually all of us. In that case, you may be able to feel normal enough to look for a new job -- especially one that doesn't require frequent or risky travel.

Who knows, one day you may look back on this bad situation and come to regard this employer as having done you a favor!

MOrfinger Message
20 May 2014, 01:43 PM

As with many others who have SCLS, I've had a very successful experience with IVIG which had been approved by my private insurance company. Now that Medicare is my primary, I am being told that this "off-label" treatment won't be approved. Do you know of any successful Medicare appeals for IVIG use and can you offer any advice?

Answer

Your question is very relevant because all of us are aging and likely moving to new health plans for retirees which are usually government-provided, so continuity of therapy and other transition issues will emerge and we will have to deal with them sooner or later.

I am aware of only 2 patients in the USA who have transitioned to Medicare: one in Florida had no problem and his IVIG treatments continued to be covered; the other in New Jersey was denied IVIG treatments for SCLS, but since he has another hematological condition which can be treated with IVIG, his physician pursued and obtained approval for it for that other condition. As a result, he too is getting his IVIG.

The problem with U.S. Medicare is that decisions like these are not made at the national level: there are 15 regional contractors who administer the Medicare program, and thus a patient in New Jersey can be told something different than someone else in Florida.

Therefore, there is a chance that you will be denied treatment with IVIG, in which case you will have to mount an appeal. The good news is that an appeal can now be made on a much stronger basis than ever before, because there are something like a dozen articles about the success of IVIG in treating SCLS patients who are otherwise not episode-free, and your own medical history is supportive of it, too.

In addition, I know that Dr. Kirk Druey of NIH, the scientist who has been working with our community for the past 6 years, and who is currently working on an article on the success of IVIG for SCLS patients, is willing to write letters of support for everyone he has seen and who meets the criteria for IVIG therapy.

Therefore, I suggest that you enlist the help of the physician who knows your medical history best in order to file any petition or appeal, and if he/she needs any help in presenting the articles that have been written, he/she should consult the Disorder Resources section of this website, and also write to Dr. Druey, in order to prepare the strongest possible appeal with his help.

We wish you a successful transition into Medicare.

SusanPeterson Message
23 Mar 2014, 11:52 PM

I was diagnosed with SCLS in 2004. I am so nauseated from the Theophyline and Turbutaline. Dr Kapoor at the Mayo Clinic in Rochester MN stated to me that an IVIG therapy may cause kidney failure in me. I still get severe joint pain and flu like symptoms. The edema is such a struggle, also. Just finding clothes that fit: I went from 125 pounds to 300 pounds in 2 months' time during my first attack in 2004. With a lot of PT and a change in diet and meds, my weight declined to 198. I would appreciate any suggestions . Thank you.

Answer

People with SCLS typically have strictly temporary gains of weight depending on the amount of fluid they take or are given during the course of an episode, but rarely do they gain and then keep a higher weight. However, there are various other rare illnesses that generate the buildup in edema and weight that you describe.

I suggest that you get the doctor who knows you best, or else Mayo's Dr. Kapoor, to refer you for a thorough consultation with Dr. Kirk Druey and his team at NIH, kdruey@niaid.nih.gov, tel. 301-435-8875. If you don't have SCLS, they are best placed to tell you what you really have -- and how to treat it. If accepted, your stay at NIH is free of charge. All you have to do is get yourself there (in Bethesda, MD, right outside Washington DC).

cmanmommy Message
2 Jul 2013, 12:09 AM

Has anyone noticed that this condition causes people not to be able to handle exposure to heat/humidity for extended periods of time?

Answer

Well no, I have not heard or read about that, but I'm not sure just what you mean by "not being able to handle" exposure to heat/humidity.

We have a number of members of this community who live in warm and humid areas (e.g., in the south of the United States) or are exposed to warm and humid summers, and they haven't written about having to live in an airconditioned habitat to, say, prevent episodes of SCLS..

markc Message
27 Dec 2012, 10:02 PM

I've read conflicting recommendations regarding whether I should get a flu shot or not now that I am on IVIG. I know that IVIG reduces the effectiveness of vaccines, but I would like to know if you or others who are also on IVIG get flu shots or other vaccines, and if so, when do you get them relative to your IVIG treatments.

Answer

To my knowledge, most SCLS patients have been encouraged by their respective doctors to get, and have been getting, flu shots for years now without any harmful effects -- regardless of whether they are or are not receiving monthly infusions of IVIG.

In the Discussion Forum "Similarities in Attacks," a couple of patients wrote that they experienced problems when getting their flu shots days after receiving their IVIG infusions, and one wrote that he has subsequently always had his flu shots before, rather than after, his IVIG infusions.

For my part, in the past 3 years that I have been on IVIG, I have chosen to receive my flu shots a week after getting my IVIG infusions -- I figured that my body was better protected against threats, including vaccine-related threats, after receiving a recent dose of IVIG -- and I have experienced no problems of any kind.

SHANNON67 Message
20 Oct 2012, 02:47 PM

I just got out of the of the hospital after spending 2 weeks with another episode of SCLS. They put me on water restriction, no IV fluids and doubled my Theophylline intake when it was already at 18.7. I need help because I just recently moved, and my old hospital knew me and my case, but this one doesn't. Can you help me to find the contact information for the NIH?

Answer

If you are on high doses of Theophylline and are still having episodes of SCLS then you are a prime candidate for switching to IVIG therapy. Try to meet with a senior immunologist, internal-medicine or hematology doctor, preferably one that is affiliated with a medical school, at your new location to discuss such a treatment plan. Point them to the reading materials in the Disorder Resources section of this website and encourage them to contact Dr. Druey at NIH right away, whose information appears at the bottom of the material in the Disorder Details tab of this website (Dr. Kirk Druey, kdruey@niaid.nih.gov, tel. 301-435-8875.) Good luck!

kbottisti Message
8 Oct 2012, 09:05 PM

Our son was diagnosed with having leaky capillaries with no known cause. We are looking for any input as to what direction we should go in next since the doctors who have been treating him feel they are against a wall. He was born with down syndrome and 2 heart defects on July 6, 2012. Any information you have would be extremely helpful and appreciated.

Answer

I'm terribly sorry to hear about your baby's multiple medical problems. I suggest that you have the doctors contact the experts at the National Institutes of Health, starting with Dr. Kirk Druey, who knows a lot about leaky capillaries and can put them in touch with other colleagues, if necessary. His contact information is at the bottom of the page on Disorder Details in this site.

rose44 Message
13 Sep 2012, 01:11 AM

I am having trouble getting my insurance to approve treatment with IVIG due to costs and having intense episodes while I wait without treatment. Thank you for your help.

Answer

I am very troubled by your message because I would like to think that, in this day and age, any duly diagnosed SCLS patient who is still having episodes should be able to get private or government medical coverage for IVIG infusions.

When I began this community back in 2009, it was a real struggle to get coverage for IVIG infusions, not only in the USA but in many foreign countries, but by now just about any patient who wants it is able to get it if he/she -- and their main doctors -- are willing to make a properly documented case before their health-care organization, and to file whatever appeal is necessary to any misguided, initially negative decision.

After all, numerous medical studies have been published in the last 3 years showing the benefits of an IVIG therapy, see the Disorder Details section in this website, especially when other treatments have failed.

Please write to me at aporzeca@american.edu and I will send you a "CARE package for you to pass on to your doctors. Better yet, have your doctors read the articles in the Disorder Details section and have them contact Dr. Druey at NIH for advice on how to proceed. It would be helpful to your case if you were to have your doctor make an appointment with Dr. Druey's team for an evaluation of your case. See the very bottom of the Disorder Details section of this website for how they should go about doing that.

MOrfinger Message
5 Sep 2012, 03:06 PM

I have had 3 episodes of SCLS, though none in the past year since I've started an IVIG therapy. You mentioned that you have permanent disabilities in your feet & hands. I have nerve damage creating numbness in both feet -- the toe areas in particular. Do you know of anything to cure this numbness? Do the damaged nerves regenerate to a degree or has the disability in your feet remained at the same level?

Answer

The nerve damage that many of us have experienced in our limbs is the result of past episodes of SCLS, during which our plasma leaked into muscle compartments and crushed our nerves and muscles. The damage is greatest when we were given extra fluids to keep our vital organs alive, much of which leaked out, and also when we were not operated early on to relieve such pressure via fasciotomies -- the cutting open of our muscle compartments.

In principle, nerves are capable of regenerating, but in practice that depends on how badly and for how long they were crushed. In my case many nerves and muscles were crushed to the point of permanent destruction, especially in my lower legs. I suffered less damage in my arms, so some of the nerves going into my hands recovered within the first year or so, especially with hand therapy and exercise -- but others never did.

Nerve regeneration is a very slow process, so you should be patient. However, if you don't notice an improvement with the passage of time -- say, after a year or two -- chances are that you suffered permanent damage.

It is vitally important that we all protect and check daily on our insensitive areas -- especially the toes and feet -- to make sure that they remain 100% healthy. An undetected wound in insensitive toes or feet (e.g., after trying on new shoes, or after walking barefoot on unclean surfaces) could easily lead to an undetected infection that might spread out of control within days, necessitating the foot's amputation in a worst-case scenario.

f2fnok Message
5 Sep 2012, 01:39 PM

I question whether or not my husband has this disorder. He has had several episodes of unexplained severe fluid retention, been hospitalized and the last time this included renal failure. The cardiologist states that he must not be following his diet since the body does not produce its own fluid. Is there absolute testing for diagnosis? I have heard that people with this disorder almost always have a high level of protein in their blood (some say this is the diagnosing factor)? What advice can you give me?

Answer

As you will read in the Disorder Details section of this website, Systemic Capillary Leak Syndrome is diagnosed based on symptoms and the presence of an unusual protein called a Monoclonal Gammopathy of Unknown Significance (MGUS) in most patients (over 80% of total).

The symptoms for a diagnosis are hypotension (a temporary drop in blood pressure), hemoconcentration (a temporary increase in the concentration of red blood cells), hypoalbuminemia (a temporary drop in the level of albumin) and the presence of the MGUS. And by temporary we mean a period of 2-4 days.

Have your doctors read the primer on SCLS published in 2010 by Drs. Druey and Greipp in The Annals of Internal Medicine, titled "Narrative review: the systemic capillary leak syndrome." It is the first article listed in the Disorder Resources section of this website. The article shows doctors how to make a proper diagnosis of SCLS.

Community User List

Diagnosed with Systemic Capillary Leak Syndrome after several years of attacks starting around age 46. I'm currentl 51, residing in St. Louis, MO, US.

I also have Meniere's Disease, slight...

Trying to self-diagose and help my doctors figure out what has caused 10 hospitalizations in the last 5 years related to fluid retention. Possible SCLS.

The first episode occurred in April 2019. After an upper respiratory infection, after flying for four hours, the feet were swollen and low blood pressure.

 In May 2020, after a long-distance...

amj

46 year old female, no children, healthy life and no previous health problems.    Several members of maternal family with rare degenerative and hereditary illness.

Mid...

Mother to son who has capillary leak syndrome and has been successful with ivig treatmwnt for 10 yrs 

Mother to son who has capillary leak syndrome and has been successful with ivig treatmwnt for 10 yrs 

Hallo, ich bin Deutsche und 59 Jahre alt. 40 Jahre mit vielen unklaren Symptomen / Krankheiten (Monoklonale Gammopathie, Sklerose, Fibrose...), extreme Schmerzen und vieles mehr. V.a....

Diagnosed with SCLS in February 2021 after what was probably my third attack in 18 years. The trigger the third time was the Moderna COVID vaccine. I am grateful to the doctors who diagnosed me...

My daughter passed away from Clarkson's disease, or so we believe. I hope to get a diagnosis for her post-mortem and to help further understanding of this sydrome.

After 15 years of semi-diagnosed illnesses, my allergist identified I likely have a varient of Systemic Capillary Leakage Syndrome. So I am new to this diagnosis (which at least is more in the...

Sas

Mother of a daughter (16y) who might have SCLS (Netherlands)

 Diagnosed in March 2011 following 3 Clarkson attacks.
 Prise en charge par ivg et suivie par le Pr Levesques du CHU de Rouen.
  

Born with IgA immunodeficiency, two episodes of Idiopathic Systemic Capillary Leak Syndrome (Clarkson’s Disease). 

I was diagnosed CLS in September 2019 while I was on holidays in France. At first stage I was treated in the hospital of  Brest (region of Bretagne in France)  later on I was...

Ik heb mijn eerste en enigste aanval gehad in januari 2017. Ze hebben toen mijn beide benen en armen moeten openen om de druk van mijn ledematen af te halen. Door de aanval heb ik ook blijvende...

Hi, I am a 48 year old woman. I live in Brisbane, Australia. I was originally given a clinical diagnoses of ISCLS in January 2019. Since then I have been under medical investigation...

Diagnosed SCLS on February 21, 2019 at 61 years old

  • This is my husband's SCLS story:
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I was diagnosed with SCLS following a second SCLS episode, the first I was in the ICU unit and ventilated/induced coma, at that time the doctors thought it was sepsis. The first episode was in...

Systemic Capillary Leak Syndrome - what's this thing that's taken over my body??Otherwise normally superfit 52 year old. Happy to be involved in any and all research, etc. Frequently travel...

Having read what I wrote here while confined to my hospital room on day three of my current stint for very low hematocrit values (<2.5) (July 2021)
I realize how atrocious what I had...

My husband was diagnosed with SCLS in 2018 after 2 events- one in January 2018 and the first in December 2016. Both events began with flu-like symptoms and he quickly went downhill into acute heart...

Systemic Capillary Leak Syndrome Patient. Ex USAF/Airline Pilot.

I have recently(September 2018) been diagnosed with scls after a 4 week stay in hospital, most of which in intensive care. I had severe swelling around my body but mostly my legs resulting in the...

SCLS - Perth, Western Australia, 28 Male

First attack, Started around 17/11/2017, SJOG Midland Hospital

Flu like symptoms and masssive swelling in legs, guts and arms. Drove to work 12 hrs but...

I have had SCLS for over 8 years and been doing IVIG since June of 2012 checking in first time when I first got this over 30 doctors said I would not survive I am crippled in three of my limbs...

I am a nurse who have a Clarkson syndrome evolving since several years.
At first, the symptomatology was chronic, but gradually, crisis became more and more severe (since October 2017). 

...

I was diagnosed with SCLS in 2018 after many hospitalizations and was started on IVIG in February 2018.

Husband to a patient that is highly suspected to have SCLS as of March 2018 - her doctors have been in contact with Dr. Druey, and he indicated it seemed like a classic presentation of...

Critical care physician

I was diagnosed Jan. 2018 .I had an acute attack and ended up in ICU . Fortunately I was diagnosed within three days . I am on no medication and am trying to get an appointment with an immunologist...

I was diagnosed with SCLS in early February of 2018. I suffered my first major leak in December of 2016. The cardiologist at Johns Hopkins at that time saved my life, but did not have a...

I was diagnosed with SCLS in Boston in early 2000 - almost 3 weeks after initially being admitted to the hospital. I felt flu-like and completely lethargic the day I was admitted, to the point...

L

Just diagnosed in Nov 2017 with SCLS. I am 48 from Canada.

I had my first episode in January 2017. At this time I thought I had the flu virus and didn’t think much of it. After a few days I...

I have been recently diagnosed with smoldering myeloma and edema due to capillary leakage syndrome. The build up of fluid started last spring. I have been put on oral steroids which seems to take...

arielbatt@yahoo.com

Update to October 2018: I have been a little over a year with recommended dose of IVIG, I get tired more than usual, but less than a few months ago, I continued with my...

 Wife of the Arielbatt patient and Argentine physician

Started having attacks 2007. Diagnosed after near death experience 2009.

Married to Ruth with three little scoundrels,Sofia, 10,Luke, 6 and Dan, 5. 

Diagnosed in Nov '14 with a devastating attack which required extensive surgery and a 6 week stay in the...

I've been dealing with what appears to be a chronic form of capillary leak condition for several years. It started after a viral infection and became chronic over time. All of the usual causes...

Test profile

Chronic capillary leak syndrome

I have been diagnosed with this disease for the last 4 years, and although all at present is going well I would like to be able to talk to others who have this.

 

I live in the Uk and would be...

Italian /Spanish hotelier diagnosed with a chronic presentation of SCLS on January 31st, 2017.

Daily maintenance treatment since 1/31/17: Terbutaline 30mg, Spironolactone 200mg, Bilastine...

Dott.ssa Pollet Cristina-Internal medicine doctor-that work in an Day service Internal Medicine Ward in S. Giovannni Battista Hospital of Turin, North-west Italy
student

Hello my name is Andrea, I am 45 years old and from Germany. I recieved the diagnoses of Capillary leak Syndrome in march 2016.

Was diagnosed in 2010 at Mayo Clinic by Dr. Phillip Greipp. 

First attack 1998 (18 more attacks before diagnosed. First in 98, next not until 2004, increased in frequency until 2010 for total...

? Clarksons
I have been recently diagnosed with systemic capillary leak syndrome. My first "episode" put me in the ICU for 3 days. Since then I have been hospitalized twice and several smaller episodes. I have...
Developed Systemic Capillary Leak Symdrome this past Winter when I had the flu. I have been quite ill past 4 years with Mast Cell Disease. The SCLS has now shifted from a chronic form to an acute...
*33 year old, struggling with suspected SCLS since August 2015. Attacks mild to moderate rather than severe, but occur every week or so. Previously very active person with reasonably good health...
Having had cyclically hospitalising attacks over the last 5 years I have finally been diagnosed with clarksons as all other test are negative . Like all of you I'm sure have had extensive tests and...
I am here because a relative of mine was recently diagnosed with SCLS. Am locating resources for him.

test profile

Diagnosed in the year 2000 at Mayo Clinic (Minn). I live in Florida and am followed by doctors who know very little about capillary leak, I do travel to Mayo occasionally in hopes of updates with...

I am married to Cristiano, my husband was diagnosed with SCLS february 2016. We live in Denmark with our 2 children (10 and 8 years old).

 

• First episode:

 

October...

Mother of a daughter diagnosed with FMF and SCLS

Born 1974, live in Germany near Stuttgart, married, have two young Kids, damaged legs (fcopmartment syndrome, asciotomy) and feet, fine motor skills of my hands are bad (criticall Illness...

I am a volunteer podcast organizer for RareShare.
I have just been diagnosed with SCLS. I am 23. Attending college at Florida state college at Jacksonville. Been sick for about six months, almost died a few times. Glad my hematologist figured it...
I am 42,married to Ruth and have 3 monsters living in my house called Sofia,Luke and Daniel. My wife tells me we have to care for these monsters until they become human.

 

Keen sports fan and...
May have had Clarkson's Disease for 2 and 1/2 years. Have almost certainly had it since October 2015. Am being treated by Dr. Druey and by the medical faculty at the University of Florida. I have...
I'm 33 years old.Im from Tehran/Iran but lately moved to Dubai/UAE

 

But I due to my condition I go back and forth.

 

3 years ago I had a rough time in my life a year full of stress,

 ...

recently diagnosed with SCLS and feeling a little lost..
ASDASDASD ASDSDA
65 yo male, diagnosed in Jan 15 after weekly bouts of hypotension, hemoconcentration, hypoalbuminemia,edema, severe abdominal pain. 1st episode was Oct 2014 with massive edema, cardiac arrest,...

My first attack was 3/2000. RAK amputation, nerve damage in both hands & left foot. In hospital for 4 months.

 

  • Second attack 4/2005. Was in hospital for a week, but thankfully no...

Hello,

My name is Annmarie and I am a 57 year old female and live in Sydney, Australia.

I am suffering from a proposed mild but very chronic version of Capillary Leak Syndrome. I suffer...

Daughter has SCLS

Female Age 48 - very fit and healthy previously . Experienced first episode Sept 2014 and been having repeat attacks on average 1 per month ( but nor clearly linked to hormonal cycles) since then....

My daughter-in-law has recently been diagnosed with this disease. I want to find out as much as I can in order to understand what the disease does to her, and what I can do to help her
hard worker
My name is Marlies and Im living in Potsdam, Germany. I experienced a very critical episode of SCLS in March 2015. Although I have permanent disabilities in my lower legs I was lucky enough to...
Donnie had his first episode in Jan '08. He was in Columbus GA. He was admitted released and aemitted again over a 10 day period. He gained 30lbs and was treated for rhabdomyolysis. He had his...
My son Levi is 2 1/2 and we are pretty sure he has SCLS.
Je suis atteinte de lupus érythémateux dissiminé depuis l'âge de 11 ans. Depuis 10 ans, j'ai développé du psoriasis et le symdrome d'hyperperméabilité capillaire.
Diagnosed at 11 years old.

 

first attack at 8 years of age. It took us three years to get a diagnosis. Has been on IVIG since February 2015 and is doing very well, no attacks since Juli 2014
My Brother Guy Allen Overland has been diagnosed with Capillary leak syndrome since 2003
Hello

 

I need treatment-informations for one of my famaly-members

 

in france,

 

who has the "Systemic Capillary Leak Syndrome"

 

i didnt find the home community on your web-site...
Diagnostic since 16/12/2014
My wife was diagnosed with SCLS Oct. 2014. That was her third attack with severe cerebral involvement (brainstem, hippocampus, thalamus)

 

 

2009 is when she had her first episode after a...
I had a diagnosis two months ago (October 2014) for seems a mild and chronic form of SCLS. By remembering what happened since January 2014, I realized that I was probably ill as soon as February,...
Hi my name is Enny. My daughter is 5 and was loosely diagnosed with SCLS earlier this year when she was still 4. This month will mark one year since my daughter fell sick and ended up in trauma...
32 year old mother of two from Rowlett, TX. Diagnosed in September 2014, a year after onset of symptoms. Looking for solid advice and info on doctors in the Dallas area with any knowledge of this...
I'm currently casting a medical mystery docu-series for the Discovery network.
I was diagnosed with SCLS Jan. 2014. July of 2013 is when I had my first episode. I had flu like symptoms and excruciating pain in my right lower back area. I couldn't straighten out my leg cause...
Previously fit (extremely) GP. I fell ill with my first attack on the day I retired, in July 2014. I am not sure how I survived the first attack with an Hct of 219, sudden drop in renal function,...
I have recently been diagnosed with Clarksons Syndrome also known as Ideopathic Systemic Capilliary Leakage Syndrome.

 

I would like to join your community to find out more about it and add my...
I'm a young doctor who's studying idiopathic systemic capillary leak syndrome, or Clarkson's disease.

 

I work in Milan, at L.Sacco Hospital, with prof. M. Cicardi. We're conducting medical...
Medically retired hospital executive diagnosed with Clarkson's Disease, pituitary disease, secondary adrenal insufficiency and autonomic dysfunction.
I'm one of the social media interns for RareShare. I help raise awareness for rare diseases and rare disease support groups
My friends husband has Systemic Capillary leak Syndrome
Hi- have scleroderma, polycythemia, dermatomyositis, allergic angioedam and hashimotos thyroid. Am still working and enjoy my work. Life is hard and would like to be a member of some groups where...
I am in fourties & survived an attack of SCLS this year in Feb. I suffered compartment syndromes with its complications requiring fasciatomies. I am now going through rehab, getting monthly IVIG &...
49 Year Old Male.

 

Living Sydney Australia

 

Diagnosed with SCLS April 2014
I have Clarkson disease and struggle everyday. I have total body edema along with joint pain. Also suffer from flu like symptoms. I have been treated in Rochester Mn at the Mayo Clinic. I started...
I live near Annapolis, Maryland. I was diagnosed with SCLS in March of 2014. That was my 3rd attack in 15 months, survived the other two on my own pretty well, but didn't know what was going on....
My daughter was first diagnosed with Capillary Leak Syndrome in 2006. However, she had other symptoms that didn't fit the condition, so after nearly 2 weeks in ICU, she was discharged with primary...
hi
I am new to the community. I just received my diagnosis a couple of weeks ago. My head is still spinning and I am trying to read everything I can get my hands on. I have been prescribed waist high...
friend of a person with scls
Pathologist
I am a capillary leak patient and have never had the opportunity to speak with anyone else who deals with the problem. I would like to connect with others willing to share their experiences. I must...

I am the mother of a wonderful 12 year old girl .She has systemic capillary leak syndrome. 3 severe episodes requiring hospitalization. Being treated with sub Q igg therapy. Hoping to connect with...

Our son Connor has had three acute episodes from March 2009 to Nov. 2011 between the ages of 4 and 6 1/2. He hasn't had one since.

 

 

My wife, Jen, is also a member of the site, but I...
For years I have been looking for answers to my medical issues. The closest diagnosis that my drs and I have come up with is SCLS/Clarksons. After looking into hereditary angioedema. I am still...
A caring friend of someone I've known for over 30 years. I'm trying to learn what I can to understand their situation better and hopefully help as I'm deeply shaken by this all.
My 8 year old son has just been diagnosed with Systematic Capillary Leak Syndrome. We live in Sydney Australia, I am scared to death of this disease, but it does help to read how everybody copes...
SCLS diagnosed 2008 Jul
hello,

 

I am a french woman who suffers capillary leak syndrome.

 

Doctors diagnosed two weeks ago .

 

I would like to discuss with you because there is no forum about this ill in...
I live in India ( Rajasthan) with my father and mother. My father is a retired officer , 64 yrs of age.He had always been healthy and with no ailments. Yet last year in April he suffered from...
To Come
my brother in law is suffering frm the ailment hence would like to join the community
Hi I am relative of Mr. Rahul Bali who is a Patient and is presenlty in ICU
I am a close Relative of Mr. Rahul Bali Who is Suffering from This rare disease

     My name is Kimberly and I was diagnosed with Idiopathic Systemic Capillary Leak Syndrome in January of 2012 by Dr. Carl Lauter at Beaumont Hospital in Royal Oak, Michigan....

Parent of a young man with Clarkson's.
My boyfriend and I have a total of three children, his daughter has just been diagnosed with SCLS. Well, maybe not diagnosed, but that is what NIH has decided she probably has after lots of testing...
I research symptoms that my husband suffers and read about possibilities that I can then take back to the doctors. Latest is systematic capillary leak syndrome. He has been told repeatedly that the...
44 y.o. single parent - developed scls 8 months post op status post mesh placement 2/2010 - systemic shock severe issues began 10/2010 - mesh removed 11/2011 - immune system damage...

Just in the centre of the Netherlands I live in Nijkerk. We have 4 boys.

Until my 50th I seemed to be healthy. I had a very busy and inspiring job as general practitioner. I loved all kind of...

My mother died in 1992 from SCLS . I am curious about new developments in treatment of this illness, and if there have been any causal factors that might have been identified.

 

We have a...

 

A first occurrence took place on 25th of October 2005 and it will supposed to be interned in the ICU (intensive care unit) of HOSPITAL UNIVERSITARIO RIO HORTEGA in Valladolid (Spain) where I...

I am 53 living in Paris , France.I discovered in May 12 I had the SCLS and my doctor is Pr Amoura at Hopital Pitié Salpétrière in Paris .

 

Before that my first "crisis" started in summer 2010...
I am a retired high school principal.
I am the mother of scls patient, Marilyn Meaux, or maire602
I am a christian mother of two beautiful little girls. I am very loving and have a gentle and kind heart.
Hi Everyone,

 

After just returning from 4 days in the hospital (my 5th episode) in So. CA while visiting my daughter & her family, I have decided it's time to get serious about finding help...
My mom suffers from SCLS and I'm working hard to help her find the best treatment possible to prevent her from having any further episodes. I appreciate this community and am excited to learn from...
My friend has been diagnosed with capillary leak.
Father of a patient.
My little brother (34 years old) has been gone through two SCLS crisis in last two years (Feb and oct 2012). He is now taking IVIG once a month and its been a while with no other hospitalization....
..
Diagnosed at University Hospital, London, Ontario, Canada by Immunologist (now retired) over 20 years ago, still alive, and have an episode on avg 1/yr. Interested in treatments, experiences, etc
Sister -in- law of SCLS victim who passed on Feb. 19, 2011. We would like to spread more awareness about this disease.
I am the proud mom of two beautiful children. Last year my world felt like it was crumbling around me when my 4 year old son was diagnosed with Capillary Leak Syndrome on his 2nd admission to...

40 year old, mother of four, diagnosed on Dec. 8, 2011 with SCLS. Hospitalized in ICU for 5 days, 4 of which were spent in an induced coma, intubated. Fasciotomies were done on all four extremities...

I am a 44 yr old sahm to identical twin boys. I was diagnosed with Capillary leaking syndrome 9 years ago after many unexplained "attacks" of what the doctors thought was me...

Husband & advocate of Marilyn SCLS patient 1/2008 6wks LCMH, ER, ICU, floor, physical rehab. 11/2008 mild case,12/2008 4wks ST. Lukes Episcopal Houston Texas, 1/2009 Mayou Clinic Rochester,...

50 Years old

 

Male

 

Living near London in the UK

 

Married with 3 children (all boys)

 

I was a research Biochemist for 12 years and am now a teacher at a secondary school

...

My 4 year old nephew was recently diagnosed with SCLS. He has had four episodes in the past 12 months - one in January 2011, one in March 2011, one in June 2011 and one in Dec 2011. Each episode...
Bonjour,

 

J'ai un problème lymphatique depuis la puberté (1950). Hyperperméabilité capillaire de 20% avec oedème généralisé. Le Pr Lagrue (Mondor-Créteil) a diagnostiqué un oedème cyclique...
*scls

 

Have had CLS for 4 Years first time was in I C U for 11 days my Doctors did not know what was wrong .

 

My blood pressure and count dropped. So they said I was in dehydration gave...

My first episode was in 2008. I was misdiagnosed with anaphylaxsis up until fall 2011. I started IVIG treatment in February 2012. Since then, I have had no episodes. I am now on bi-weekly...

My daughter Ela (3 this November 2011) was diagnosed with SCLS, September 2011, after her last serious attack.

 

 

Ela has had 4 episodes in the past year (September 2010 to date). 3...
I was told that I have chronic capillary leak but that I do not fit into the strict category of SCLS because I do not have an episode resulting in an ICU stay or proven hemoconcentration. I am...
One of my best friends is suffering with this disorder, and I wanted to find out more about it.
In early July, 2010 (the hottest weekend of the summer) I arrived at the emergency room with a low pulse, rapid heart rate and profuse sweating. When they checked my blood, they saw what they...
My name is Amy and I have been just informed I have Clarkson's Disesase. I am a mother of 3 and grandmother of 5. I have been battle with this for over 21 years and finally came across a doctor at...
*I was diagnosed on June 24, 2011 by Dr. Griepp. My story: In Feb. 2011 I was having swelling first in my hands and then spread to my feet and legs. Went to my Primary doctor. She ran some test for...

I had my first acute attack of CLS around 10 years ago and again 3 years ago. My most recent attack was around two months ago and it was very severe. This last time I was diagnosed with CLS. The...

I have been diagnosed since April 2011

 

I was a puzzling case 3yrs ago due to showing similar symptoms to MS all test came back negative,

 

I was discharged after two weeks with right...
friend of someone who died from scls
I am a family member of Mary Jane K, hoping to get more information on this disease.

I was diagnosed in 1998 with SCLS. I had been ill for 6 months, and gained 80 lbs. I was given prednisone too prevent an allergic reaction to a test. It worked wonders, and I lost the fluid. I...

i was diagnosed with scls in November 2005 or actually 3 months later in February 2006. After I had emergency open heart surgery to put a window in my heart sack at age 38. In a coma for nearly two...
I am at retirement age and have suffered from an auto-immune disease for the past 15 years.
My sister Shannon suffers from SCLS; diagnosed 5 years ago (2006).

 

 

Shannon has just been admited into Cardiac Care to monitor her current episode. We have been fortunate to have the...
I am a 61 year old female. I started swelling when I was 14 years old - just a week after my father's sudden and unexpected death. After his funeral, we went to visit family that could not attend,...

Previously healthy individual - jogger and active in excercising. First episode 9/24/10. Thought I had the flu. Extremely dizzy, nauseated. Doctor came over and couldn't find pulse. Put on 30...

wife has capillary leak syndrome

Sept 4, 2010 - Persistant Flu-like symptoms for several days led me first to my doctor's office and then to the Emergency Room ICU. I was admitted with extremely low blood pressure. I was put...

I am a retired family physician with MGUS and have had progressive symptoms of recurring edema, low blood pressure, near syncope that may be a chronic variant of SCLS.
I was diagnosed with SCLS in December of 2010 after suffering multiple episodes for over a two year period.

 

 

I was initially put on Theophylline and Prednisone with one minor recurrence...
I am the husband of someone with (?) SCLS and/or angioedema
my 4 yr old son was dagnosed with capillary leak syndrome. he has had 3 episodes in the last 6 months and thie last episode led to a cardiac arrest. he was put on life support and luckily recovered...
Physician brother of a member
I'm 19 years old, living in Oklahoma. I'm in my second year of college, and am double-majoring in Forensic Science and Funeral Service. I enjoy playing the piano , guitar, and singing; going to...
As new member but with diagnose of SCLS in April 1988 after a severe collapse and a first hospitalization in distress and 3 more other until January 1989, I think I will, probably, be at time the “...
My first attack was 3/2000. RAK amputation, nerve damage in both hands & left foot. In hospital for 4 months.

 

Second attack 4/2005. Was in hospital for a week, but thankfully no physical...

I have been diagnosed with systemic capillary leak syndrome which I have had the symptoms of for about 9-10 years. I am seeking information and other people who also have this as I live in New...

Diagnosed by the Mayo Clinic in Rochester , MN , with SCLS in August 2011. This was my second trip to Mayo. The first trip was in August 2010 and resulted in a 2 week stay and a suspected / working...

I have just been diagnosed with SCLS -8/18/2010 at the Mayo Clinic in Rochester, MN by Dr. P. Greipp. My first episode of this disease was June 2007. I played a round of Golf and had eaten dinner...
Wife of Mahaff

I have SCLS and think I am one of the oldest persons with this disease in this group. First attack, May 2007 after severe bronchial infection, 8 days in ICU in an induced coma; 14 days in hospital...

I was diagnosed with Systemic Capillary Leak Syndrome in May of 2010. This was after 3 months of extensive testing of every kind that did not explain the fluid retention that I had. I was first in...
My cousin has been diagnosed with this rare disease.
Atypical Systemic Capillary Leak Syndrome (current working diagnosis) Diagnosis made this year at Mayo Clinic in Minn. I am taking Theophylline and had my first IVIG a month ago. I first recognized...
I am a 56 year old female who has suffered from systemic capillary leak syndrome for 10 years. I leak almost constantly but my episodes are relatively minor with my largest 24 hour weight gain at...
Surprise! I've got a rare disease! I'm still going to be a pain in the butt, though.
Searching for answers that no doctor has been able to find in over 5 years for my wife. In the beginning she was having severe body aches, menstrual problems, severe acid reflux. After having a...
My daughter, Rebecca, was diagnosed with SCLS in 2007, when she was 2 years old. I've joined this SCLS community to learn more about this illness and its symptoms and treatments, and to share our...
High,

 

 

My name is Wout and my mother is diagnost with the clarkson capillary leak syndrom. We just recieved this diagnose 3 days ago two weeks afther she had an attack and had to go to...
Dear sir

 

I have the pleaser to write about my casI`ll try to explain about may case .

 

All start at 17/02/2008 I had a cove and I went to the emergency they give me anti biotic it was...
My medical history briefly. It began in June 1995. After several months of hospitalization, I was diagnosed with SCLS of November 1995, immediately started plasmapheresis with a transfer of plasma...
my extremely good friend was diagnosed 6 years ago and I am agressivley seeking information to assist him. We live in Longview, Texas.
seeking helping in treatment of my 16 year old daughter newly dx of idopathic systemic capillary syndrome

My husband's first episode:

  • Husband and I were ill with upper respiratory infection (confirmed to be Human Metapneumovirus).
  • Husband had to be hospitalized Dec 2009 
  • He received a least...
I have suffered SCLS since 2007 and have had two near fatal attacks. My condition is currently being successfully controlled with Ventolin and Theophylline tablets, and I have had one attack in 18...
I am the mother of a 5 year old with systemic capillary leak syndrome. My daughter was diagnosed with this disease at the age of 17 mo. She receives infusions of IVIG, 25g every four weeks, and is...
Our son Connor (born 4/7/05) was diagnosed with this syndrome on Christmas Eve 2009. This was his second episode in 9 months. His first one (March 2009) was when he had the flu and was misdiagnosed...
I am an infusion nurse who worked very briefly with Judy Davis and I would love to read the article on your website. Thanks.

 

 

I'm Judy Davis' sister
My sister, Judy Davis, died on November 27, 2009, after a five-year battle with SCLS.

 

 

Bonjour,

 

Un urologue d'un service hospitalier m'a diagnostiqué cette maladie rare il y a quelques années.Le traitement proposé à l'époque, me faisait plus souffrir que la maladie...

 

Je...
My father has just passed away from possible systematic capillary leak syndrome. He was not diagnosed with the condition eventhough he suffered from it more then once.

 

I suppose I'm on a life...
I am a gastroenterologist working at the Department of Gastroenterology of the Ospedale V. Cervello, Palermo, Italy.
My sister-in-law, Denise, has SCLS. She is married to my brother, Mike The picture is of their daughters, Kate & Brooke. My nieces! They are wonderful! Denise was diagnosed in 2009. And, with...
I've been diagnosed in 2006 with Idiopathic Capillary Leak. It is a severe case that has been constant since December,2004.I am being treat by a "Kidney-Doctor" and I'm on 20-mg of Lasix2x/day.My...

first attack in 02/2009. the doctors find the exact diagnosis after 24h and in the meanwhile, I had two fasciotomies in the legs. quick recovery and no server damage in nerves and muscles. The...

I am a 61 year old male living in Decatur Al. I had my first major attack in 3/05, with 2 more in 07, and 08, 3 in all. I was diagnosed in 4/07 at Vanderbilt in Nashville,Tn by a research doctor,...
I am the wife of a Systemic Capillary Leak Syndrome patient. I am interested in corresponding with other patients or family of patients.
Parent to a daughter with Systemic Capillary Leak Syndrome
I am french, I understand english but I don't write In french now Je suis malade SCLS depuis avril 2008 pendant 1 an j'ai eu des perfusions d'immunoglobuline et maintenant tous les 2 mois 1g/kg...
My name is Maria, I was diagnosed with SCLS in Feb 2011. I have the chronic form of SCLS with attacks every few days. I had my 1st major attack in January of 2004 at the age of 33, but I started...
Update 2016:

 

 

I have been off IVIG since February. For now I am on Singulair and an ACE inhibitor. I have been having minor attacks but have been able to stay out of the hospital.

...

I am a stay- at- home mom of 7 children, (5 sons and 2 daughters).

 

And yes, they are all ours,

 

no, we have no twins, and

 

no, we are not Catholic.

 

These are always the first...

[Updated on January 2013]

 

My daughter is 7 years old and has been diagnosed SCLS in May 09. She had 4 SCLS attacks, the first one in February 08, the last one in April 09 (she was 3-4 yrs...

My daughter, age 41, has been battling illness for almost 2 years now. It appears that she has systemic capillary leak syndrome, a very rare condition. We are very anxious to learn all we can about...

I noticed swelling in my ankles in spring of 2007, which increased through that summer. I was working full time at a desk job, then on evenings & weekends at our home business helping with...

family physician , patient with scls
My first attact was in Oct 2005. I was disgnosed in June 2006 with Capillary Leak Syndrome. The first time I entered Mass General in Boston Mass the Dr's thought I had a virus due to the fact that...
I was just diagnosed with scls and am looking for any information on it, since my doctor was unable to give me much of anything yesterday. So I am taking it upon myself to do some of the research...
sono la moglie di mario gatto se qualcuno in italia vuole contattarmi per avere informazioni sulla malattia puo' farlo su facebook mi chiamo marcella iannacchero
I live in Christchurch, New Zealand, and if I could find some way of connecting with some supporting people I would be very grateful.

 

 

I have been living with SCLS since October 2005. I...

Name: Walt Breidigan, Bethel Park, Pa. Born 1957

 

 

I have had CLS since February, 2005.

 

 

The first incident gave me two total drop feet and no feeling from calves to the...

Heading a research study on Systemic Capillary Leak Syndrome at the National Institutes of Health.

I have been on IVIG therapy for 5 years without any SCLS episodes.

I have had 7 recurring episode of Severe Capillary Leak Syndrome in the past 20 years. My last episode was in January 2013. Each...

Widow of NC SCLS patient
I have had SCLS since 1997, It took 6 years to get it diagnosed. Aftyer it was diagnosed I went into remission for 5 years. After coming down with a systemic viral infection in the fall of 2008 my...
my brother at age 43 suffered a capillary leak attack and was diagnosed 6 months later in 2004 at the mayo clinic in rochester by dr greipp and his associates. i was with him both times. he is now...
White male diagnosed with Capillary Leak Syndrome at the Mayo Clinic in Rochester MN after a severe illness in Nov. 2003.
Im from Guatemala. The MD told me that my mother have capillary leak syndrome, but here is not a specialist. I need help. I prefer if can write me in spanish. Thank you.
My name is Claude Pfefferlé. I live in the french speaking part of Switzerland.

 

First acute episode of systemic capillary leak syndrome on May 2003. Failure of different treatments and 5 new...
j'ai été diagnostiqué avec le Syndrome de fuite capillaire depuis Mars 2006. j'ai pris des médicaments durant deux ans sans aucune rechute. En Janvier 2008, ça recommence... des hospitalisations...
I am from Canada, My friend, 38 years old, mother of two beautiful childrens has this desease since 2006, and presently her situation is not controled. She is desperate to find a way to controle...
Am an Indian national, want to join this community on clarkson's syndrome to know more about it, its treatment, cures if any and what prophylactic measures can taken.
I AM AN INDIAN LIVING IN KANPUR( UTTAR PRADESH) & I HAD 2 SEVERE ATTACKS SINCE 2007 (& HAD TO BE ADMITED TO ICU FOR RECOVERY), ALONG WITH 6 MINOR ATTACKS SINCE 2004 , AS PER MY DOCTOR . I AM DOING...
Physician focused on disorders of blood pressure control and sodium metabolism. Some experience with Idiopathic Systemic Capillary Leak Syndrome. Based at Weill Cornell and The New York Hospital...
First scls attack on valentines day 2005. Many minor attacks and hospitalizations untill beggining IVIG therapy March 2010 I believe. Life changing cessation of symptoms until October leading to a...

My husband Jeff was first diagnosed with SCLS when he was hospitalized in February 2005. We're in Washington, DC. He's been hospitalized numerous times since then.

Friends, family and patients with SCLS

 

 

I am a physician at the Mayo Clinic in Rochester MN. I have been interested in helping patients with SCLS for many years. I am happy to support...
Mark is my name, I am the partner of my lovely girlfriend Cara, who has recently been diagnosed with SCLS.

 

 

She has had a difficult time recently, with admissions to ICU and the upset...
I am 36 years old and was diagnosed with SCLS in January of 2007. The first episode I had of this was quite severe and landed me in the ICU on a ventilator and in a medically induced coma. I was 11...
I have had this syndrome since June 2007. I was diagnosised in Dec. 2007. I was having episodes about every 5 to 6 days. I had a severe episode in November of 2008. After a bad episode I would...
I am married to Richard and we have 2 wonderful sons 17 and 20 years old. I have been a Hopkins pediatric infusion nurse for almost 30 years. I enjoy church, reading, cooking and my family. I...

I had my first episode of what turned out to be SCLS in November 2005, and was very lucky to have survived it (though with permanent disabilities in arms and legs, and thus in hands and feet) and...

My name is David Isserman and I am one of the co-founders of RareShare. If you have any questions or feedback regarding RareShare, please feel free to contact me directly at david@rareshare.org.

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