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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 seven 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 died in March 2011; Bruno Galien (bruno) from Nord-Pas-de-Calais, France, who passed on in February 2012; Guy Allen Overland (allenoverland) from the Washington DC area, USA, who died in January 2015; Marilyn Meaux (maire602) from Louisiana, USA, who passed away in March 2017; Julie Eady (jodono) from Perth, Australia, who died in September 2017; and Cara O'Hagan (Cara) from Dublin, Ireland, who passed on in February 2018.

 

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 seven 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 died in March 2011; Bruno Galien (bruno) from Nord-Pas-de-Calais, France, who passed on in February 2012; Guy Allen Overland (allenoverland) from the Washington DC area, USA, who died in January 2015; Marilyn Meaux (maire602) from Louisiana, USA, who passed away in March 2017; Julie Eady (jodono) from Perth, Australia, who died in September 2017; and Cara O'Hagan (Cara) from Dublin, Ireland, who passed on in February 2018.

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

Less than one in 1 million people are affected by this disease.

Name Abbreviation
Clarkson or Clarkson's Disease Clarkson

Systemic Capillary Leak Syndrome (SCLS) is idiopathic, and 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, hemoconcentration, hypoalbuminemia, and the presence of a protein called Monoclonal Gammopathy of Unknown Significance (MGUS).

Diagnostic tests of Systemic Capillary Leak Syndrome has not been added yet
  • Methylprednisolone 125 mg IV STAT, repeated as needed.
  • Judicious use of IVF boluses and drips to keep CVP above zero.
  • Phenylephrine or Norepinephrine for hypotension, early institution.
  • 50 ml of 25% albumin, repeated as needed.
  • Continuous CVP monitoring, stat and serial lab work including CPK and lactate.
  • Immediate Orthopedics consult and compartment pressure measurement; early, preventive limb fasciotomies if compartment pressures or CPK high. 
  • Venous Doppler for DVT, may need full anticoagulation.

Treatment of a fully developed SCLS episode requires recognition that there are two phases. The first phase, which often lasts a couple of days, is called the resuscitation phase because the dual aim of ER/ICU treatment is to stop or control the capillary leak and to raise the patient's blood pressure from near zero. In this initial phase, an albumin and fluid leak from the capillaries into the tissue spaces causes swelling, especially into the extremities rather than the abdomen or organs (such as the lungs). The blood pressure falls and the red cells concentrate. This loss of fluid has similar effects on the circulation as dehydration, slowing both the flow of oxygen carrying blood to tissues and the output of urine.

Glucocorticoids (steroids like methylprednisolone) are recommended to reduce or stop the capillary leak, and albumin and colloids usually help to increase the remaining blood flow to vital organs like the kidneys. Keeping up with the fluid loss is important because sustained low blood pressure can damage vital organs such as the kidneys. Even though blood pressure readings may reach and remain at very low levels, it is important to avoid overly aggressive intravenous fluid administration causing massive swelling of the extremities.

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 may well 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: β-agonists like theophylline and terbutaline, and a prophylaxis with IVIG infusions. In recent years, more and more patients have been migrated from the former to the latter in Canada, Europe, the United States and beyond, because IVIG therapy leads to superior results -- no episodes or fewer and lighter episodes than compared to no therapy or the other therapies -- and does not have as many adverse side effects as does treatment with β-agonists like theophylline and terbutaline.

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). There is growing evidence that IVIG (usually, 2 gr/kg per month, administered over two consecutive days) has worked for many patients in Europe for over 10 years now, and is proving extremely successful among patients who have tried it in North America and beyond in the past several years, thus having become the standard of 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. 646-962-2605,
email mpecker@med.cornell.edu ;

in Europe:
Prof. Zahir Amoura,
Département de Médecine Interne,
Hôpital de la Pitié-Salpêtrière, Bd. de l'Hôpital 83, Paris 13e,
tél. 0033 1 42 17 80 81,
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. Linda Scott at LMScott@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. Those who wish to be greeted in person during their stay by this community's founder, please contact aporzeca@american.edu

 

Please see the Disorder Resources section.

FINALLY!!! My first IVIG Created by ValeriaSpain
Last updated 24 Jun 2019, 10:53 PM

Posted by Rita Wood
24 Jun 2019, 10:53 PM

Hi,

I received my IVIG every two weeks. I still have reactions but not near as bad as the every four week method. My kidney numbers vary as I also chronically leak. I am starting to think I have minor attacks every three or four months it I can handle them without a hospital visit. When my blood pressure is low but not a bad attack I receive extra fluids before treatment. My main problem is small intestine damage.

I hope all goes well for you.

 

Posted by ValeriaSpain
24 Jun 2019, 09:03 AM

IVIg Update

Hi!

I’m happy to share that I finally started receiving the monthly recommended 2g/kg IVIg dose.

Since my kidneys are somewhat fragile due to the recent severe attack followed by the several mild flares I suffered, my doctor decided to extend the IVIg on a fortnightly basis in lieu of 2 consecutive days. 

I received the first half on June 19th, 50 grs infused in 7 hours with no other side effects rather than a slight headache. I did sleep for several hours upon the infusion though. Preventive side-effect prophylaxis was administered prior to infusion.

In the other hand, the second half, (the other 50 grams), will be administered on July 4th, followed by the first half of the second infusion on July 18th.

Last but not least, I will fortunately -and finally- be discharged from the hospital on July 19th, after 2 long and intense months of a happy-ending story. :) 

Have a lovely start of the week and never ever lose Hope. 

Valeria

Ps.- Arturo, thanks for your kind reply about contacting Prof. Amoura and/or his team. I spoke to him on the phone in order to extend the importance of his opinion and he asked for my doctor to send him an email to better discuss, however, he did not respond to any of our emails. Luckily, Dr. Druey did reply in a matter of 3 days. A heartfelt Thank You Arturo, for your restless endeavor in helping and guiding everyone at this forum. 

Posted by aporzeca
10 Jun 2019, 10:23 AM

Valeria,

If you tried to contact Dr. Amoura directly without success, I suggest that, instead, you have your main physician contact him by email requesting an urgent consultation on your case, to include a summary of your medical history and the specific questions that he/she have for him right now.  Some of our physicians are not as comfortable responding to inquiries received from patients or caregivers as they are to inquiries from fellow physicians.  Lorena's experience with Dr. Amoura may not be typical because she is both a physician and a caregiver to an SCLS patient, and she probably identified herself to him as a medical professional.  In addition, if I were you, I would go ahead and schedule a regular appointment to see Dr. Amoura, because Paris is not that far for you and he has received our patients from throughout the European Community in the past.  Alternatively, your physician may approach a knowledgeable colleague of Dr. Amoura in his same department and hospital, such as Dr. Marc Pineton de Chambrun, see https://www.aphp.fr/offre-de-soin/consultation-37726 and email marc.pinetondechambrun@aphp.fr

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root canal and other surgeries Created by mberry
Last updated 24 Jun 2019, 07:42 AM

Posted by ValeriaSpain
24 Jun 2019, 07:26 AM

Hi!

I’m so happy to find that none of you was affected by the surgeries you went through.

I’m having a couple of root canals in about a month from now, a few days after my IVIG infusion. Since I receive my 2g/kg of IVIG split on a fortnightly basis in lieu of 2 consecutive days, I wondered if this kind of surgery occurring in between the first and the second infusion would be of risk.

I will certainly brief my dental surgeon on my condition too.

Have a lovely start of the week!

Valeria

Posted by WazzaACT
17 Dec 2018, 07:38 AM

Great

Posted by claude53
16 Dec 2018, 08:59 PM

Well played !!!   Merry Christmas to everyone !!!

Claude

 

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Physicians in Maryland with a background in rare diseases/syndromes? Created by DudeSCLS
Last updated 13 Jun 2019, 12:54 PM

Posted by WazzaACT
13 Jun 2019, 12:54 PM

Hi Kevin. While nothing can be guaranteed in life, as a survivor of 3 major CLS attacks and havining been on IVIG for 8 years with no further attacks or any particular increased risk to community diseases I think you should rest easily. I do also have my annual flu shots of course. Good luck and enjoy your life. BTW I am also supported by a GP with the characteristcs described by Arturo and a Cardiologist, Immunoligist and Hematologist all of whom are deeply aware of my condition and support me fully. I am, I recognise extremely fortunate. The other side of this is that with the IVIG my consultations have become less frequent and my health in general good. 

Posted by DudeSCLS
13 Jun 2019, 11:00 AM

Thank you for your research and input. I have met with Dr. Druey and I am very familiar with Johns Hopkins. It was there that my life was saved twice. SCLS specifically occurs to my heart. I am well known by the cardiologist at Hopkins. I have some of their personal phone numbers and have follow up appointments with them. But they treat me and check in on me as a heart failure patient, making sure my ticker is ticking fine. They’ve never encountered my condition before. You went beyond answering my question and I am very grateful. I will definitely look into the doctors you recommended at Hopkins. I really need to have confidence in the IVIG. Flu season has become the most freightening time of year for me. The cardiologist that have witnessed my SCLS have confidence so I should too. I would like my primary physician to have more knowledge on rare syndromes though. Thank you again so much for getting back to me!!

Posted by aporzeca
12 Jun 2019, 11:45 PM

Welcome to our community, Kevin!

The best physician to have on your side is a veteran internist or allergist/immunologist with a teaching and/or research appointment in a medical school -- someone who is competent AND compassionate and not afraid to manage a rare-disease patient. I looked up many of the physicians affiliated with the Frederick Regional Health System in and around Frederick and wasn't impressed by what I saw.

If I were you, I would make an appointment with someone affiliated with the Johns Hopkins School of Medicine in Baltimore who is very senior and, though they may not be a right match for you, they would be able to recommend one or another colleague interested in rare diseases, blood disorders, and immunological issues.  As a first point of contact, I like the profiles of the following Hopkins internal-medicine physicians: Drs. Eric B. Bass, Daniel Ernest Ford, Craig Evan Pollack, or Gregory Paul Prokopowicz, see https://www.hopkinsmedicine.org/gim/clinical/jhoc.html  I also like the profiles of the following Hopkins allergy/immunology physicians: Drs. Antoine Azar and Jody Robert Tversky, see https://www.hopkinsmedicine.org/allergy/interests/  Take information on your medical and hospitalization history with you to the first encounter.

If you haven't done so already, ask your current doctor to send your medical history to, and request a consultation with, Dr. Kirk Druey at NIH/NIAID, so you may visit with him and get his evaluation and best advice. He's the world's leading expert on SCLS, and you are very close to Bethesda, so it would be silly for you not to have him in your corner, as well.  His contact information is on the page Community Details, under Tips or Suggestions.

And please fill out your profile page (https://rareshare.org/profiles/dudescls) with as much detail as possible about your medical history.  We're still learning a lot from one another.

View Full Thread (1 more posts)
Most recent attack Created by gandcburns
Last updated 10 Jun 2019, 07:04 PM

Posted by ValeriaSpain
10 Jun 2019, 06:33 PM

Dear Cristina, 

Thank you very much for the update and I truly hope you finally tolerate infusions side effects ASAP.

Currently, I find myself in a very challenging situation since my doctors do not want to institute IVIG anymore upon stopping the infusions due to the intense common side effects I experienced on my 3rd initial infusion, (A daily 0.5g/kg infusion in 5 days was programmed. I weigh 50kg without edema), which I believe are a mere consequence of receiving a lower dose and a higher speed than the documented by Dr. Druey. 

So far, I am doing my best from the hospital to get my doctors contact both Dr. Druey and Prof. Amoura. It is a quite frustrating situation. 

I continuously experience an important lack of breath, dizziness, peripheral edema and extreme fatigue. The same reoccurs every three to four days when I suffer a mild episode along with drastic albumin level decrease, hypotension, +3 to 5 kg edema and high hematocrit - Arturo: I do not experience an hematocrit and Hgb increase in every episode, but in some. I use to be at 38%Hct/ 13-15Hgb and I can go from 41 to 51%Hct and 15-18Hgb in different episodes.

Moreover, I find it interesting that most “chronic leakers” we do not always experience an Hct and Hgb increase. 

Irrespective to the above mentioned, I would like to share the following case report (August 29th, 2018) in which Dr. Druey states the SCLS chronic presentation existence based on experience: 

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6119378/

Regards,

Valeria.

Posted by gandcburns
10 Jun 2019, 12:14 PM

Thank you for the advice and information Arturo. Being a heavy exerciser, I am anemic and do have low hemoglobin levels.  My doctor said the same as you about my Hb levels and wasn't too worried because they were in the normal range. It was the lightheadedness, blurry vision, and shortness of breath on day two coupled with the upward trend in the Hb level that pushed us to make the decision to admit to the hospital. Those symptoms, for me, have only accompanied the 2 full blown attacks I have had in the last 8 years. Never have I felt short of breath or dizzy with one of the smaller leaks. I have asked about the Hemocue machine, and hope to purchase one. I will discuss the Prednisone tablets with my doctor as well. 

Posted by aporzeca
10 Jun 2019, 11:06 AM

Cristina,

Many thanks for the update and here are some comments which may be helpful, though please remember that I am not licensed to practice medicine, so the following does not constitute medical advice, but just things for you to discuss with your physician. First, be more careful before categorizing potential leaks as an "attack" or even "episode."  Normal levels of hemoglobin for women are 12.0 to 15.5 gr/Dl, see https://www.mayoclinic.org/tests-procedures/hemoglobin-test/about/pac-20385075, so unless you have levels that are unusually low to begin with (e.g., because you are anemic), levels on the order of 15-16 are not necessarily indicative of a capillary leak, and minor fluctuations of the kind you mention (15.6 to 15.2 to 15.8) are usually not statistically significant.  (Hgb readings naturally fluctuate at least that much during one's normal day.)  Second, you might want to start measuring your Hgb levels on a regular basis, in order to get a baseline range that works well for you (say, 14.5-15.5), and especially so whenever you don't feel normal, so you can spot and document the difference, which should be significant and sustained (e.g., you are a 13-14 type of person and suddenly you are registering readings in the 16-18 range).  For this purpose, you should consider purchasing a HemoCue Hb 801 device, for example from https://www.medicaldevicedepot.com/HemoCue-Hb-801-Hemoglobin-Analyzer-p/hb1promo.htm or from https://www.cliawaived.com/web/Hemocue_Hb_801.htm, and all that goes with it (cuvettes, lancets, etc.)  You may have to order it through your doctor, because it's not FDA approved for patient use, and it may not be reimbursable by your insurance company -- but it could save your life and, to be sure, it will help you identify a true episode of SCLS before having to go and wait at the ER to find out that it is or it isn't.  Third, the side effects you felt from a dose of 110 gr of IVIG versus what your body is accustomed to (65 gr) are probably related to that doubling of the dose and to the infusion speed -- and not to the switch in brand from Privigen to Gammagard.  And fourth, your doctor may agree to prescribe you Prednisone tablets for you to keep at home and take whenever your Hgb reading is somewhat above normal, and before coming into the hospital, because sometimes minor episodes of SCLS can be aborted by taking a large dose of such a corticosteroid, so this is another suggestion for minimizing unnecessary visits to the ER.

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Stem Cell Therapy Created by Shannoncourt
Last updated 30 May 2019, 08:57 PM

Posted by rnolan
30 May 2019, 08:57 PM

Hi I had a stem cell transplant October last year (2018). This was done because my ISCLS had turned into Myeloma. I am in the process of cutting down on my IVIG,  however it’s been two months now and I am not sure it is working as over this last month I have felt like my legs have been swelling? My haematologist is monitoring me closely through this process so I guess only time will tell. 

My Myeloma is now back to smouldering after a year and three months of treatment which consisted of roughly 8 months is chemo, stem cell transplant and then three months of consolidation chemo. 

Its wonderful to feel well again.

Ruth (NZ)

Posted by krogers
30 May 2019, 09:43 AM

There was a published case some time ago where it was used and successful (would have to look up the reference).  From a theoretical point of view it does make sense (to me at least) as it effectively removed your own immune system ands replaces it with stem cells.  Given that as far as we know SCLS is causes by some aberrant aspect of the immune response it makes sense that it could/should work.

However stem cell transpants are very risky and a complex procedure - is it worth the danger when there is a very good, far less risky treatment i.e. IVIG.  Stick with the tried and tested treatment.

Posted by aporzeca
30 May 2019, 08:12 AM

I agree with Anthony.  Moreover, going to Colombia for stem-cell therapy strikes me as a particularly exotic suggestion.  I recommend that you have a physician you trust get in touch with Dr. Druey at NIH to ask his opinion on the matter.

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Good news Created by alapenna
Last updated 24 May 2019, 04:32 PM

Posted by aporzeca
24 May 2019, 04:32 PM

Thank you for the encouraging update on Adele!  You are all very brave for experimenting to see if children can "outgrow" SCLS.

Posted by Arielbatt
23 May 2019, 01:30 PM

Thanks for the update, the exchange in this community is very important.

Posted by Nhan Nguyen
23 May 2019, 05:19 AM

Thanks for your update! Please check my email. Best luck to everyone!

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Remission Created by prima2007
Last updated 24 May 2019, 04:18 PM

Posted by aporzeca
24 May 2019, 04:18 PM

Thank you for your encouraging update on Noah!

Posted by prima2007
24 May 2019, 06:38 AM

You can also email us at elamar@telus.net 

Posted by prima2007
24 May 2019, 06:24 AM

Our son was diagnosed in 2010 at the age of three after episodes with no diagnosis.  It was finally Dr. Anne Junker in Vancouver Canada that diagnosed him with SCLS.  He had another significant episode which nearly took his life, nearly full organ failure and we were told by doctors to prepare for the worst.  They needed to give him an immense amount of fluid to save his life but in turn put his life at risk again as this caused compartmental syndrome in his arms and legs.  He was rushed into emergency surgery and orthopedic surgeons performed fasciotomys all 10" in length, 2 on each leg and one on each arm, drains were put in, he was on a ventilator and remained in ICU for 2 weeks.  My wife and I didn't leave his bedside for a single moment, we were terrified but reassured he was strong and fighting for every breath.  He had a number of blood transfusions while in ICU but then quite quickly started to turn the corner.  He was moved to a ward and his recovery and rehabilitation began. He needed to learn to walk again due to the surgery's performed and he was immediately put on regular 6 week IVIG treatments which remained in place for 6 years.  We have just celebrated 3 years in March and his last IVIG treatment and he is doing exceptionally well.  We of course monitor for swelling and other symptoms as a common cold or flu has been whats been most dangerous for him.  We are extremely hopeful for the future, hes now almost 12 and is your typical rambunctious and somewhat "challenging" pre teen!  If you are in a similar situation as us or have just received a diagnosis or are in the middle of some difficult times...please don't give up...keep fighting, engage your doctors, your health authority anyone that will listen and give you more of a voice.  As we know there is no cure...YET!  But we are learning more every day on how to cope and manage and move forward as best we can.  Please reach out if you have questions, comments, concerns and we will do whatever we can to help to share our experience and make this journey a little less scary and lonely.  We are a strong community fighting for the ones we love.

Question Created by rnolan
Last updated 24 May 2019, 09:27 AM

SCLS Diagnosis? - UK, Birmingham Created by rebeccajane
Last updated 23 May 2019, 05:40 PM

Posted by AnthonyL
23 May 2019, 05:40 PM

Hi, Rebecca,

I was tentatively diagnosed with Mastocytosis too for almost a year by a local doctor, despite negative tryptase, normal urine histamine levels and other markers for Mast Cell disorders. I even had a bone marrow biopsy which was negative for any abnormal mast cell activity. I was also tested for a slew of other conditions, including hereditary angioedema, leukemia, heart failure, ischemic heart disease, kidney failure, liver failure, low protein, autoimmune disorders/allergies, infections, etc, which are all conditions that could cause systemic swelling symptoms like mine. All negative. I took cromolyn, antihistamines and two other meds designed to help suppress mast cell activity but none of them worked. Cromolyn seemed to help a little with intestinal issues but not much. I still had systemic edema every day. I always feel very dehydrated, as well, despite the fluid retention and I live on liquids exclusively due to intestinal swelling (my abdomen is usually grossly distended to the point of looking like a preganant woman so have no appetite). On good days, I urinate frequenty, abdomen will deflate and I drop about 10-15 pounds of fluid within a few days, but the whole cycle starts up again day or two later. I am never truly free of edema but it does wax and wane. I'm very disabled from this condition.

Anyway, I went to the Mayo clinic in MN last year and saw a hematologist there who has published on SCLS and he diagnosed me with chronic capillary leak syndrome and recommended a course of IVIG to see if it would work. Depite the fact that I don't exihibit so called markers for the condtion like an M spike and overt hemoconcentration, he still diagnosed me with the condition because my capillaries are leaking and I am third spacing fluid systemically. I am hypovolemic as well (low blood volume) and my pressure drops from normal of 120/80 to the 100/60 range when I am very swollen. Worst reading was 90/50 when I went to the ER years ago for breathing issue, swelling and dehydration.

SLCS is diagnosis of exclusion so if they've exluded all other known causes of third spacing then that's a diganosis you and your doctors should seriously consider pursuing.

I am in contact by phone with two other members of these boards who have been diagnosed with the chronic variant of SLCS and both are on IVIG for it. They were both misdiagnosed for many years and became very ill and disabled from the condtion. One of them almost died from it and has permanent disabilities because his doctors took so long to figure out what going on with him. I myself have recently developed pulmonary issues from years of living with this disorder. I am hypoxic when I lie down at the doctor's office now (oxygen readings in my blood dip very low when I lie down on by back). My immunologist thinks that it's due to the leaks/swelling. She suspects that my distended abdomen is putting too much prerssue on my diaphram and lower lungs restricting my breathing or possibly that fluid is now leaking into my lungs. She referred me to a pulmonologist and I'll know more when I see him in a few weeks. I have also developed abnormal EKG readings which nobody can figure out the cause of (my echos are still normal and an angiogram shows clear arteries). Cardiologist suspects it's from the swelling. 

I'm still waiting to get on IVIG to see if it will help me. My insurance is giving me a very hard time about covering costs so my doctor is trying to get in contact with several of the companies that manufacture IVIG to see if they can help out.

At any rate, I hope you get some answers soon. This is not a condtion you want to let fester over time. It will ultimately cause a lot of other medical problems down the road if it's not addressed and managed properly. Also, don't let anyone tell you that chronic capillary leak syndrome doesn't exist. It does. I am not sure if it's being caused by the same underlying pathology as the more acute version of the condition but it does exist. If doctors ever tell you otherwise, it's because they're ignorant of the condtion and unware of the literature published on this disorder. I am living proof that a person's capillaries can be in a state of chronic abnormal leak with no obvious cause. It's a grossly underfunded and under-researched disorder and in my physician's opinion likely a very underdiagnosed one too, due to lack of definitive diagnostic tests and knoweledge about the condition. Don't let yourself fall through the cracks like I did.

Also, BTW, here's a link to synopsis of a published paper about chronic capillary leak syndrome. https://www.ncbi.nlm.nih.gov/pubmed/30431430

It's the first ever published lit review on the chronic form of SCLS. Includes a case report as well.  It was published this year. My doctor called the authors of the paper and they sent her a full text version.

 

Good luck to you,

Anthony

 

Posted by rebeccajane
22 May 2019, 04:56 PM

Hi, I joined this discussion group 7 years ago when I suspected I had this condition, however without a diagnosis, I continued searching for answers and seeing all sorts of consultants for many years and was finally diagnosed with MCAS and hypermobility (probably hEDS) and thought I had the answer. However, all MCAS treatments including 6 Xolair injections have not stopped the swelling. Recently, all the specialists I’ve seen think this could be chronic SCLS (independent of me suggesting it!). I am getting more and more swelling/fluid accumulation throughout my whole body and feeling more and more unwell, so really need to find someone who can diagnose whether this is SCLS in the UK and possibly treat me if needed? I live in Birmingham, although I am willing to travel anywhere and wondered if anyone here knew of someone who may be able to help me?
Many thanks in advance and very best wishes! Rebecca 

Any else had medical history of pain? Created by DavidS
Last updated 14 Apr 2019, 10:06 AM

Posted by DavidS
14 Apr 2019, 10:06 AM

Thanks Annmarie. Oh, I wonder if Dave has had a mild form of chronic SCLS if there possibly can be such a thing. 

He certainly hasn’t suffered as much as some of the folks on this board with full blown attacks chronically.

He also uses triptans, has injections and tablets for pain. Is trying Ajovy injections, one of the migraine preventives that just became available. Not sure if it’s helping yet.

Posted by Annmarie
13 Apr 2019, 01:12 PM

Hi David.

I Have also been suffering with migraines and probable Chronic Capillary Leak since approximately the same age of 15. I am now 57. The migraines arrive for 3 days as the fluid is retrieving. I am currently improved on Hyrdacortisone which I have been taking for the last 6 months. If I can control the swelling, I do not get migraine at all. If I swell even a little bit, I will absolutely always get a 3 day migraine in 2-3 days time. I believe that while migraine appears to not be a part of SCLS for most people, I have a predisposition it to as other family members do. The Chronic SCLS seems to be a trigger. I take triptan dugs for the migraines, usually Relpax 80mg

Posted by DavidS
13 Apr 2019, 10:23 AM

Thanks Arturo. Yes, he did have a bit of a headache on the evening of day one, but not as severe as he’s used to. They will premedicate with Benadryl and Tylenol next time 

View Full Thread (2 more posts)
Community Podcasts
Title Description Date Link
Hospitals, Doctors, Medical Teams: Navigating Barriers of Rare Diseases (Patient Navigation)

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)

08/17/2018
Community External News Link
Title Date Link
Community Resources
Title Description Date Link
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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.

07/05/2017
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
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
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
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
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.

03/17/2019

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: I have had no more episodes of SCLS.

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 address is aporzeca@american.edu

 

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<p>Live in Brisbane, Austra...
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<p>Systemic Capillary Leak ...
<p>Having had CRPS since 20...
<p>Systemic Capillary Leak ...
<p>I have recently(Septembe...
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<p>My first attack was 3/20...
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Discussion Forum

SCLS Community Member Mahaff

Created by dallen | Last updated 19 May 2017, 08:30 AM

IVIG dosing

Created by Mntnhiker | Last updated 15 May 2017, 06:31 PM

Welcome to our new home

Created by aporzeca | Last updated 15 May 2017, 03:09 PM

SCLS Community Member Mahaff

Created by dallen | Last updated 24 Apr 2017, 03:31 PM

new member anyone else in UK

Created by rareshare5050 | Last updated 19 Apr 2017, 08:32 AM

New Website Format

Created by rnuara | Last updated 23 Mar 2017, 01:38 PM

Off IVIG and wondering what to do next

Created by Ritz | Last updated 27 Jan 2017, 06:35 PM

new member anyone else in UK

Created by rareshare5050 | Last updated 22 Jan 2017, 11:22 AM


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