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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 five 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; and Marilyn Meaux (maire602) from Louisiana, USA, who passed away in March 2017.

 

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 five 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; and Marilyn Meaux (maire602) from Louisiana, USA, who passed away in March 2017.

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.

Community specific tip

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@psl.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. Lauren Long at _longl@mail.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 Expert, please contact _aporzeca@american.edu_

Please see the Disorder Resources section.

IVIG every 3 months Created by papa
Last updated 23 May 2017, 06:26 AM

Posted by monkeyracing
23 May 2017, 06:26 AM

Something to watch out for with IVIG prophylaxis is the possibility of a reaction 3-5 days post infusion. I've developed a very predictable rebound phase after receiving IVIG. It could be an immune reaction, but we never narrowed it down. In any case everyone reacts differently. I just react poorly.

I was getting dosed monthly, but it was making me ill like clockwork. Now I only receive IVIG as a means of dealing with an episode of SLCS. I generally insist on remaining in hospital for 5-6 days, as I know I am going to have a reaction to the IVIG. Ironically, more IVIG is the treatment prescribed and it usually straightens me out within a few days.

Posted by aporzeca
21 May 2017, 05:45 PM

Welcome to our Community!

IVIG treatments for all sorts of diagnoses generally take place every 4 weeks or else more frequently, depending on the dosage and manner. For example, we have some patients who receive their IVIG every 2 weeks -- but then they receive half the dosis that they previously got every 4 weeks.  Look it up on the Internet and see, e.g., at http://www.usbioservices.com/specialties/ivig/treatments

And the reason for this is that IVIG is a blood product, and as the body's own blood is renewed continuously, whatever IVIG a patient receives on Day One is diluted away each day after that.  Studies show that half of the dose received on Day One is gone from the body by Week Three or Four, and the remaining half disappears very rapidly after that.  (It's not a straight-line pace of decay.)  Therefore, in order for sufficient IVIG to be always present in the bloodstream -- namely, at least a half-strength -- patients must receive additional amounts by Week Three or Four.

In sum, getting IVIG every 5 or 6 weeks might potentially be justified if the initial dose is so powerful that the patient can do with just a tiny fraction of the original amount by Week Five or Six, but I don't know of any justification for giving it every 3 months -- other than for the insurance company to save money on Levi by letting him run on "empty" for a month-and-a-half.

But don't take my word for it.  Feel free to have Levi's doctor consult with an immunologist or else with Dr. Kirk Druey at NIH, kdruey@nih.gov   He's the world's leading authority on SCLS, and in 2015 he published a co-authored article on SCLS in six children, see http://pediatrics.aappublications.org/content/135/3/e730

The authors concluded that "Four of our 6 patients were treated with IV or SCIG [subcutaneous immunoglobulins], and none had a further severe episode while on this treatment, suggesting that immunoglobulin prophylaxis (1–2 g/kg per month) is effective therapy for SCLS in children."  [The other two children could not tolerate the indicated dosis all at once, so they were switched to receiving an equivalent amount subcutaneously, over many more days, in amounts carefully designed to provide the same protection as 1-2 g/kg per month.]

Posted by papa
21 May 2017, 12:57 PM

I am the grandpa of Levi who in March of 2015 at age 2 1/2 was in ICU for 2 weeks with SCLS. He survived, thank god, but ended up having a fasciotomy on his left leg, He started out having monthly IVIG treatments but has now for the last year has them every 3 months and we are happy to say no episodes since his initial episode 2 years ago. Wondering if anyone else has been able to stretch infusions out - especially the children afflicted ?

Hemocue Created by Kimmieroumayah
Last updated 26 May 2017, 03:29 AM

Posted by Kimmieroumayah
26 May 2017, 03:29 AM

To everyone else who commented,

Thank you for you input.  I was slightly shy about purchasing such an important piece of medical equipment from Amazon or EBay.  I need to be certain I can rely on accurate results before presenting this information to my Dr.

 I cannot rely on low BP alone anymore.  My blood results are often very different from my BP.  I do not seem to have a BP lower than 98/68 which is well within the range of normal even when my blood results prove that I am in capillary leak.  So I believe that the Hemocue is my best shot at determining  for certain that I am in a chronic state of capillary leak.  

Once again thank you to all for your support 

Kimberly

Posted by Kimmieroumayah
26 May 2017, 03:17 AM

Dear Dee,

Thank you so very much for your heart felt offer.  I am still weeping for you and your loss.  I don't believe that I could possibly accept, even though it would be a blessing.  I cannot even begin to imagine how difficult this entire illness has been on you and your family.  It isn't just the individual who suffers from this devastating condition, it is the entire family who also suffer.  

Thank you for your offer and if I am unable to purchase one on my own I would be grateful for your help.  

Kimberly

Posted by dallen
23 May 2017, 08:50 PM

Hi Kimberly,

I still have my husband's (Mahaff) and would be willing to give it to you if you'd like. It's an Hb201+.  He took very good care of it, kept it in the box along with all the notes and instructions that came with it. There's also a pack of microcuvettes in the box but those are expired, you'll have to buy new ones. I used to purchase them from Amazon.

Let me know if you're interested.

Dee

View Full Thread (3 more posts)
Organ damage Created by Ritz
Last updated 23 May 2017, 06:16 AM

Posted by monkeyracing
23 May 2017, 06:16 AM

Sorry to hear of your troubles. My first episode was extremely severe. My whole body shut down. Multi-organ failure, multiple ischemias, compartment syndrome - all that stuff. There is life after a severe episode. I lost an eye, and ear and a lot of muscles and nerves (don't freak out. I'm an extreme case!) but I'm still kicking and fighting. Even with fairly frequent ER visits, I've managed to maintain and even expand on my dark sense of humour and deep sarcasm. Keep your chin up!

Posted by jisenhour
16 May 2017, 05:27 PM

Hello Ritz,

     I'm sorry you had to go through that.  I skipped the heart attack, and stroke but I did have an attack that caused part of my bowel to be removed, and two other attacks where my abdomen was opened up to check for it.  I was in the hospital for 2-4 weeks each time, and another two months to approach normal.  I do hope the IVIG treats you better this time, and there's a community that understands whet you're going through.

Jeff

Posted by tiggrrr027
9 May 2017, 08:44 AM

Hi Rita, So sorry to hear of your life threatening episode but so happy you survived. The reason my brother Allen Overland passed away from his final episode was in part due to severe bleeding from DIC ( a clotting disorder) which was due to the rhabdomyalysos from the swelling. He had never had any type of bleeding in the past and this came on quickly and the doctor was hand pumping blood into him to try and keep up with the loss. Unfortunately he had several severe strokes and that is what caused his death. I do not wish to scare anyone and I know you have had problems with IVIG but consider yourself extremely lucky!!! Dr Druey was there when all this happened with my brother so any information you can give him as Arturo mentioned will not only help you but others as well. It took some time for them to figure out what medication to give him to stop the bleeding and even though that may not have saved my brother I know Dr. Druey has that information in case it happens to someone else. Please take care and be well!! Linda

View Full Thread (2 more posts)
SCLS Community Member Mahaff Created by dallen
Last updated 19 May 2017, 08:30 AM

Posted by elganzory
19 May 2017, 08:30 AM

So sorry for your loss my condolences 

yaser

Posted by tiggrrr027
9 May 2017, 08:57 AM

So sorry to hear of your loss. Thoughts and prayers be with you.

Posted by Josephite
26 Apr 2017, 07:57 PM

My condolences.

View Full Thread (2 more posts)
SCLS Community Member Mahaff Created by dallen
Last updated 24 Apr 2017, 03:31 PM

Update On Me Created by monkeyracing
Last updated 23 May 2017, 06:36 AM

Posted by monkeyracing
23 May 2017, 06:36 AM

I had another, very severe episode last month, that had me in the ICU for a bit. I had what felt like my usual monthly flare, but I never became hemo-concentrated. I went to the ER and my usual protocol was followed. 200ml 3% saline infused over 20 minutes (deals with cerebral edema) follwed by IVIG.

I remained in hospital waiting for my body to react to the IVIG and boy, did it ever! This was probably my worst episode since the first in 2010. I've had over 70 ER visits, but this one stood out. I even got on the road to loonieville, with a few hallucinations and some delirium. The only thing that wasn't odd about it was I was able to predict when I was going to rebound 3 days earlier. I'm able to do so every single time and my doctor still never believes me. Ugh.

I'm not going to complain, though. I'm still here, so I suppose it wasn't that bad.

Posted by monkeyracing
15 Apr 2017, 06:02 AM

Also...we don't believe theophylline is doing anything for me, good or bad.

my doctor believes that I am leaking all the time and high blood pressure may be forcing fluids from my circulation.

weird.

Posted by monkeyracing
6 Apr 2017, 05:58 AM

Oops. I had written avery long post while lying in bed the other night, something weird happened and I thought it had been completely lost. I guess the title survived. Perhaps I'll just sum it up as briefly as possible.

I've been through the ER more times than I can count in the past year. My record for staying healthy was just shy of 5 eeks last year, but most months I was in more than once.Doctors have a bad habit of encouraging me to leave after a couple of days, but I always have a bounce back 3-5 days after receiving IVIG. I should know better than to take the bait when my life is on the line. Had a few close calls, but in those cases I was still admitted.

My treatment has morphed a little bit over the past few years. we discontinued the monthly IVIG treatments as they were actually causing more episodes than they prevented. My emergency protocol now involves a bolus of hypertonic saline to get my sodium levels up (they tend to drop) and create a gradient between the blood in my general circulation and that in my brain. Cerebral edema is something that's been happening and getting IVIG tends to make it worse for me. My dosage of IVIG has been halved on ER admissions, but I'm usually getting another dose when everything starts shifting after a few days. After the second, or third, dose, I usually recover fairly quickly.

As to the specifics of my case and research that's being done on my (our) behalf locally. My genetics have been checked out and nothing really stands out. I have an elevated but stable MGUS protein, as many of you do. We've done cytokine panels and have noticed one that's slightly elevated when I'm ill, but treating it would involved shutting down my immune system with some nasty drugs. My doc has been running a study involving capillary leak syndrome during pregnancy and has been making comparisons between my case (as a non-pregnant guy!) and his research pool. The testing involves a laser beam being reflected off the back of my eyes and into an incredibly sensitive scanning device. I think it's called OCT. We've learned that when fluid shifts in my circulation, my retinas either swell or thin by a few microns, or some other tiny measurement.

So, that's the not so brief briefing on what's up with me. If anyone has any questions, hit me up. I've learned a lot in the past 7 years.

Jim

View Full Thread (1 more posts)
Welcome to our new home Created by aporzeca
Last updated 15 May 2017, 03:09 PM

Posted by Kimmieroumayah
15 May 2017, 03:09 PM

Wow!   Surprised by the new site! 

So much more information and medical terms that I will have to learn.  

Posted by Beverley
24 Mar 2017, 11:59 AM

I am so pleased i found this site. I am learning so much and my consultants are listening, although i am finding this illness is so rare that no one knows what to do with me when im really poorly. I am curently out of control and having small attacks that are keeping me in bed!! Thank you so much for all the hard work and for everyone who shares their experiences. 

Posted by aporzeca
24 Mar 2017, 01:49 AM

RareShare was co-founded (and built from scratch) in early 2008 by two wonderful, idealistic men in their twenties named David Isserman and Eric Steel, on the thought that there should be a better way to bring together patients, families and healthcare professionals to share knowledge and personal experiences about rare medical disorders.  They never intended to make any money from this site -- and sure enough, they never did.

I stumbled upon RareShare in May of that year and promptly started the SCLS community, and over the past decade it has turned out to be the most successful virtual community within RareShare -- but then, that's my opinion.  Indeed, I measure success not just by the relatively large number of members (over 300) we have attracted considering that SCLS is ultra-rare, and by the over 350 informative discussion topics we have generated, but because we have definitely improved and actually saved many lives -- not just my own -- by posting very useful information on diagnosis, management and therapy options for SCLS.

In 2014, Isserman and Steel transferred the rights to RareShare to the Rare Genomics Institute (RG) and moved on with their lives.

RG is an international non-profit that provides access to cutting-edge research technologies, physicians, and scientists across the globe. By providing an expert network and an online crowdfunding mechanism, RG helps families pursue personalized research projects for diseases not otherwise studied. RG was founded by Cheng-Ho Jimmy Lin, MD, PhD, MHS, whose day job is to be the Chief Scientific Officer (CSO) at Natera (NASDAQ: NTRA), where he is leading the development of new diagnostic technologies for cancer.

From Day One, the folks at RG committed to building a new electronic platform for RareShare, because the site was antiquated, limited in its functionality, and was stagnating and prone to failure because it was written in an obsolete code that fewer and fewer people knew about. It took more than two years but they finally did it -- all on the back of volunteers, by the way.

As you may have noticed, there are still some bugs in this RareShare 2.0, but they are being addressed.  For instance, the useful Disorder Resources section had not made it through the migration from RareShare 1.0, but it's now there and I've just updated it to reflect the latest (two 2017) publications on SCLS -- scroll down to the bottom and see for yourself.

Also, the way it's been set up, we won't be getting immediate notifications of new posts; rather, emails will go out after midnight EST each day alerting us to new material -- like this Discussion Forum topic, I hope.

On the other hand, there are great new functionalities.  Now you can email another RareShare member directly, rather than posting a general message under Discussion Forum in the hope of reaching the intended party.  Also, you can now search for information across the site, e.g., you can search for "headaches" and you will see entries on headaches from all kinds of communities beyond our own.

Anyway, welcome to our new home!

Arturo

New Website Format Created by rnuara
Last updated 23 Mar 2017, 01:38 PM

Posted by rnuara
23 Mar 2017, 01:38 PM

Thanks Arturo.

Posted by aporzeca
23 Mar 2017, 01:31 PM

Yes, and I've reported it and they said they;re going to work on it.

If you start by going to the first page of the website, www.rareshare.org and you log in, then you can go on without trouble to https://rareshare.org/communities/systemic-capillary-leak-syndrome

Feel free to report it also.

Posted by rnuara
23 Mar 2017, 12:33 PM

Is anyone else having problems directly logging into our homepage for SCLS on RareShare website, with the new version?

Differences in Brands of IVIG and strengths Created by skadi1
Last updated 22 Mar 2017, 08:16 PM

Posted by skadi1
22 Mar 2017, 08:16 PM

Thankyou so much for your advice.I have requested an immunoligist many times but have been denied.On monday just gone,i had treatment and have been put on Intragram 10 %as the hospital is stopping the Intragram 6%, they gave me the same amount of gms as when i was on the 6% i put it forward that now that i am on the 10% and my weight is 76kg my dosage should be re evaluated and was bluntly told that i did not know what i was talking about and to leave it up to them,so whilst having treatment i made sure the nurse treating me was going to listen.After 2 days i got to see a resident( i think ) Heamatologist he is under my actual Heamatologist ,i cannot get to see the actual specialist no matter how hard i protest.Anyway the "resident" Dr has told me it will take up to a minimum of 6 months before i can get an appointment.Thats just not good enough for me so i have contacted 2 Goverment Health Department and put in a complaint.Thankyou for being my back up it has helped me in so many ways .Cheers Katarina : )

Posted by aporzeca
13 Mar 2017, 11:32 PM

Katarina,

I am no immunologist, never mind a physician, but I think that your suspicions are correct, so I encourage you to confront your doctors or otherwise insist on seeing an immunologist, because they know more about IVIG than any other specialist.  My understanding is that Kiovig 10% and Privigen 10% are equivalent strengths of IVIG, because they both deliver 10 g of human normal immunoglobulin for every vial of 100 ml.  However, Intragam 6% is not equivalent, because it delivers 6 g of human normal immunoglobulin for every vial of 100 ml.-- namely, 40% less immunoglobulin.  Therefore, your dosage probably should be adjusted upward by 40% to compensate you for the difference.  It could be that they reduced the dose to minimize any side effects, or to see what difference it would make, or simply to save money.

Please fill out your profile page, because it is currently empty.  Tell your fellow community members when and how you were diagnosed with SCLS; what was your medical history of symptoms, episodes, treatments and etc.; for how long you have been receiving IVIG and what medications you tried with what success before that.  We need as much information as possible.

 

Posted by skadi1
11 Mar 2017, 09:52 PM

HI : )  I am a little confused and am I
hoping someone out there can clarify something for me as I am getting conflicting information from my Drs here . Is there a difference between the strength of Kiovig 10 Privigen 10 and Intragram 6. The reason for my question is that when I started on IVIG  I was on Kiovig 10 and after a bit of time we got the dose right so my IVIG levels stayed above 20 .Then the hospital changed brands and I was on Privigen 10,again we played around and had my levels above 20 up until my next treatment . I reacted to the Privigen so i was then put on Intragram 6 . I had been give  90 gms every fortnight when I was on  
Kiovig and Privigen regardless of my weight .When I started IVIG I was 105 kg I am now 75 kg.When I was changed to Intragram the Drs decided to drop my dosage to 81 gms a fortnight and my levels dropped below 20 and I started retaining fluid  a few days before I was due for next treatment.As soon as I had treatment I would release the fluid but it made me unwell.So I asked if they could increase my dosage so I am above 20, they did and I was receiving 89 gms of Intragram 6 every fortnight and it worked a treat .Last treatment i was told my dosage had been dropped back to 81 because of my weight , I protested but to no avail . And guess what my levels dropped again.below 20 again and I started holding fluid .Dose the difference of % between these brands have anything to do with this.I have asked and been told that Privigen 10 and Intragram 6 work the same.Yet my body seems to need more Intragram to keep my levels above 20. Can someone please explain this to me because I have to convince the Drs that I need a higher dosage yet they are convinced that since my weight is  75 kgs I should recieve no more than 81 gms. Any input would be greatly appreciated . Cheeers Katarina

Hi everyone, Is the Disorder Resources Folder empty now? I found the folder really helpful in the former Homepage. Or are the documents at another place now. w? Created by AndreasGunsser
Last updated 27 Mar 2017, 10:49 AM

Posted by aporzeca
27 Mar 2017, 10:49 AM

The folder has been restored and I have added the lastest entries -- two big-picture aricles coming out this year (2017), which appear at the bottom.

Posted by aporzeca
5 Mar 2017, 04:32 PM

Andreas,

I am working with the volunteers who developed RareShare 2.0 to fix some issues including this one.  In the meantime, if you need anything specifically, send me an email to aporzeca@american.edu

Arturo

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)

03/01/2017
Community Resources
Title Description Date Link
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
The 5-Minute ICU Consult Entry on SCLS

The 5-Minute ICU Consult Entry on SCLS

Abstract: The entry on SCLS appears on pages 256-257 of the 2012 edition of this new guide containing more than 200 entries covering all aspects of adult critical care. The 2-page protocol includes the basics, diagnosis, treatment, and ongoing care of patients with SCLS.

03/24/2017
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
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: 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 another article with the results of a survey of SCLS patients who have been on IVIG.

My address is aporzeca@american.edu

 

Expert Questions

Ask a question

Gwendolyn20 Message
4 Jan 2017, 08:33 PM

My friend was diagnosed with SCLS about 2 years ago and has been on IVIG for almost the 2 years after his first episode. He now decided to stop the IVIG infusions, though he's very well aware of the risk that comes should he get a second episode. I'm just wondering if there's anything I could do to help him in this transition as I don't know what his reaction might be. Or if there should be any red flags I should look for in case he gets an episode, or any signs that he could be getting one, or should get back on the IVIG.

Answer

Until the specific cause(s) of SCLS can be identified, and a more targeted therapy can be developed, the experts at the National Institutes of Health recommend that confirmed SCLS patients should receive monthly IVIG infusions indefinitely.

If I were you, I would encourage your friend and his main physician to reach out and consult with Dr. Kirk Druey at NIH, especially if this was not done previously. (His coordinates appear towards the bottom of the tab "Disorder Details.") I would want to make sure that (1) my friend's diagnosis of SCLS was made correctly -- mistakes are common -- and (2) that an informed dialogue between knowledgeable physicians and my friend had taken place.

The upside of your friend stopping his therapy is that if by chance (a) he was indeed a confirmed case of SCLS and (b) he has no more episodes despite being off his medication, that would certainly provide worthwhile information to the rest of us who endure these expensive and intrusive treatments indefinitely.

The downside of your friend stopping his therapy is that he could have a life- or limb-threatening episode. The episodes are characterized by 3 clinical features measurable in a hospital's Emergency Room setting: hypotension (unusually low blood pressure), hemoconcentration (too many red blood cells per unit of plasma), and hypoalbuminemia (low albumin levels in the blood). (See more under "Disorder Details".)

And if he were to have an episode, tell him to demand a crash infusion of IVIG upon confirmation of the 3 clinical symptoms. (And it would help a great deal if he were to carry with him a letter from his physician stating that he is to be given IVIG if he should be having a confirmed episode.)

While there is limited experience with SCLS patients surviving an episode thanks to getting a last-minute infusion of IVIG -- and I have personally watched two SCLS patients die in the hospital despite getting their first taste of IVIG during their episodes -- at least nobody could blame themselves for not trying, in a life-or-death emergency, what seems to work best when received on a regular, monthly basis.

Rubeanie Message
29 Dec 2016, 04:15 AM

I am not sure if I am a rare breed or very fortunate (so far). In February of this year 2016), I had a "rash" from head to toe. I was misdiagnosed, but then in April I had what my doctors believe was a full-blown episode of SCLS. I have had several small ones since then. I was put on montelukast (Singulair) and have not had an episode since. My question is: will the montelukast always work, or will the SCLS flare up again?

Answer

If you really have SCLS, then you are both a rare breed -- because SCLS is a one-in-a-million, exceedingly rare disease -- and very fortunate so far -- because there is zero evidence that montelukast (Singulair) prevents episodes of SCLS.

Before the good news about IVIG for SCLS became widely known (namely, before 2008), I used to take theophylline, terbutaline and montelukast to prevent further episodes -- and yet I went on to have them again and again until I received my first dose of IVIG in late 2009. If I had not, I would probably have died in 2010.

Therefore, my advice to you is as follows. First, you must have your diagnosed confirmed, and the best way to go about doing that is to have your treating physician send in ASAP a copy of your medical records to Dr. Kirk Druey at NIH (see contact information at the bottom of the tab "Disorder Details") requesting an expert evaluation. If Dr. Druey suspects that the diagnosis may be correct, he will probably invite you to fly in to NIH (located in Bethesda, MD, in the outskirts of Washington, DC) for a full, in-person assessment.

And second, if and when your diagnosis of SCLS is confirmed, then Dr. Druey will work with your physician on a treatment plan which may well include an IVIG-based therapy.

Please do not delay: if you really have SCLS, then it's only a question of when, not if, you will experience your next episode -- and it could be fatal.

cpollet Message
20 Nov 2016, 10:27 PM

I'm a young Italian doctor that is following for about 6 months a male patient, 57 years old, for a suspected diagnosis of SCLS. His history began in 2014...

...

I'd like to know if, in your experience, I could improve his clinical situation by the addition of other drugs (Immunoglobiln iv? Terbutaline?) and if so in what dosage. My experience in SLCS doesn't exist and all that I know results from reading some literature. I ask you for help to improve my experience and my patient's health

Answer

Dear Dr. Pollet:

As you will understand, we are strongly discouraged from making diagnoses and recommending specific treatments on this website.

Therefore, I suggest that you request a consultation from any one of the leading authorities on SCLS identified towards the bottom of our page "Disorder Details": Dr. Kirk Druey at NIH; Dr. Mark Pecker at Weill Cornell/New York Hospital; and/or Dr. Zahir Amoura at the Hôpital de la Pitié-Salpêtrière in Paris.

Their emails are provided, so all you need to do is write to them giving them the details you sent to me plus whatever additional medical history you can deliver, and I am sure they will write back to you with their best advice on how to proceed.

LorraineKH Message
24 Jun 2016, 05:02 PM

A family member (nephew) was recently diagnosed with SCLS, while out of state. He seemingly received excellent care, and now I am trying to help with resource finding and financial assistance for what undoubtedly will be a very expensive trip to the hospital. I would like to be his ombudsman in the healthcare system he is in, do you have any idea how I might go about doing that? He is very intelligent, but I expect him to go through some periods of grieving and adjustment after his elation of finally coming home. Finally, are there any physicians in Colorado with experience treating SCLS? I have a bazillion questions, but will stop here for now! Thank you for being here!

Answer

Welcome to our Community! You will find here just about all the information you and your nephew need, so start by reading the main sections ("Disorder Details" and "Disorder Resources") and then going through the "Discussion Forum," picking among the topics that interest you most. You'll want to print out some of these sections and have your nephew take the printouts whenever he sees a doctor or finds himself in a hospital's ER

If your nephew was diagnosed correctly -- and especially if he wasn't -- the first thing he will need is a top-notch internist to serve as his "anchor physician." Forget about finding an expert on SCLS; you need to find a veteran, compassionate physician who will make the time to manage a rare-disease patient, is willing to read up on the condition, and is pro-active in consulting with the handful of physicians who really know about SCLS, as mentioned in the "Disorder Details" section.

My advice is to look for one who is affiliated with a medical school in Colorado (preferably, the Univ. of Colorado), and has the rank of Professor or at least Associate Professor, as long as they practice within, say, a couple of hours driving distance from where your nephew will live. Check out the list at http://www.ucdenver.edu/academics/colleges/medicalschool/departments/medicine/GIM/Faculty_Staff/Pages/Faculty-Roster.aspx

And please encourage your nephew to educate himself about his diagnosis and to join this Community. Many of our members, myself included, are alive and have a reasonably good quality of life thanks to what we learned here -- and only here. Besides, it's therapeutic to interact with others who provide information and moral support.

davec Message
21 Jan 2016, 07:38 PM

As I recover from my bouts with SCLS, I am curious if exercise in the gym, jogging or tennis is recommended? Second, how long after an episode should one wait before traveling internationally again: 6 months, a year?

Answer

As far as we know, physical exercise and international travel do not cause episodes of SCLS.

We have a few patients in this Community who were very active (e.g., biking or skying) for decades before they had their first episode of SCLS, and then we have a vast majority of patients who exercised moderately or little before they were hit. Likewise, we have a few patients who used to do a lot of domestic and/or international travel before their first episode, and a large proportion of patients who did not travel much.

Of course, we also have many patients who would like to engage in moderate or vigorous exercise and sports but cannot because of the damage they suffered in feet, legs, hands, arms or vital organs in the wake of their first episode of SCLS.

We also have patients who would like to travel abroad but are afraid to do so because they don't want to fall ill far away from home, family and their medical doctors.

In this regard, consider yourself very fortunate to be able to contemplate re-engaging in sports and in international travel.

The question you should ask yourself is: Why am I still having bouts of (presumed) SCLS? Being vulnerable to further episodes is the most risky thing you can do, because if you really suffer from SCLS, and you are not receiving a therapy that is effective in preventing the episodes, then you are vulnerable to having an episode that will leave you crippled or dead.

If you haven't done so already, I recommend that you have your physician send your medical history to Dr. Kirk Druey at NIH, see bottom of the "Disorder Details" section, so that he and his team may confirm your diagnosis and work with your physician to come up with an effective course of therapy for you.

janiegrrrlaz Message
9 Dec 2015, 07:04 PM

Are you aware of any treating physicians in the Phoenix (AZ) area? I was turned away by both the Mayo Clinic here and in Rochester, MN. I was told they do not "have any one qualified" to treat me. I am waiting to hear back from NIH also. Thank you!

Answer

I'm sorry that the Mayo Clinic seems to have dropped the ball in your case, but you really don't need them.

While I don't have the name of any physicians who have treated an SCLS patient in Arizona, I have two common-sense suggestions good for anyone in any place.

First, make absolutely sure that you have been diagnosed correctly by having your medical and hospital records sent by your main physician to Dr. Kirk Druey at NIH, requesting a consultation on your case. It sounds like you already initiated contact with him, so that is good. Having a correct diagnosis is crucial to everything else.

Second, if your current physician is not interested in your case, or seems overwhelmed by it, then find a new "anchor" doctor, preferably a seasoned internist on the faculty of a university hospital, to provide a fresh pair of eyes, accept a diagnosis of SCLS, discuss your case with Dr. Druey, coordinate your care, and decide on therapy options including IVIG.

It doesn't matter that he or she may not have even heard of SCLS before: by now there is plenty of useful literature, see the Disorder Resources section of this website.

In Phoenix, you are lucky to have the University of Arizona Medical College, so I would look there. Ask for an initial appointment with the Chair of Internal Medicine, Dr. David Wisinger, tel. 602-344-5027, davidwisinger@email.arizona.edu If he doesn't take you on himself, he will have recommendations for you.

mweston Message
25 Jun 2015, 03:13 AM

I am currently battling breast cancer, and my oncologist has recommended me for a clinical trial involving T cells. It involves taking my blood, removing the T cells, growing them and coating them with antibodies, and then infusing them back into my body so they may fight cancer. The purpose of contacting you is that one of the drugs to be given to me during this trial, Interleukein-2, supposedly can trigger the Capillary Leak Syndrome! Have you heard of this connection? Should I participate in this clinical trial?

Answer

I know nothing about this, and my suggestion is that you have your oncologist contact Dr. Kirk Druey at NIH to ask him what he knows and thinks about it. He knows more than anybody about SCLS and he has a lot of top-flight colleagues he can consult with. His contact information appears toward the bottom of the "Disorder Details" tab.

Tvaughn Message
24 Jun 2015, 12:20 AM

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. Are there any treatment options that we can use in or near Dallas, Texas?

Answer

Welcome to our Community, and the best thing you and her doctors can do is read the information appearing in the "Disorder Details" and "Disorder Resources" sections of this website -- because when it comes to exceedingly rare diseases like SCLS, the lack of accurate and timely information can be disastrous for the patient and her caregivers.

And of course there are great care and treatment options available in or near Dallas, TX. I suggest that she find herself a senior internal-medicine physician affiliated with a top hospital like the Baylor Univ. Medical Center or the Methodist Dallas Medical Center.

Blinski Message
31 Mar 2015, 10:50 PM

I am a physician and am quite confident that my father, who has been hospitalized a number of times in the past 2 years, has SCLS. His symptom complex and lab results at times of attack, and difficult recoveries due to aggressive fluid resuscitation, are literally a textbook case. His current physicians are failing him miserably, so have you any suggestions of any others in the South Florida area that could help? Also, how do you find a physician that is willing and able to initiate IVIG infusions?

Answer

So sorry to hear about your father! It is bad enough to be sick, but it is worse still to be sick and without an appropriate diagnosis -- never mind a suitable therapy.

While I don't have the name of any SCLS expert in South Florida, I have two suggestions good for any place. First, get hold of your father's medical and hospital records and send them to Dr. Kirk Druey at NIH requesting a consultation on his case.

Second, find a new "anchor" physician for your father, preferably a seasoned internist on the faculty of a university hospital, to provide a fresh pair of eyes, consider a possible diagnosis of SCLS, discuss his case with Dr. Druey, coordinate his care, and decide on therapy options including IVIG. It doesn't matter that he or she may not have even heard of SCLS before: by now there is plenty of useful literature, see the Disorder Resources section of this website.

While immunologists are obviously very familiar with IVIG therapies, and one of them could be enlisted to support the initiation of a course of therapy for your father, the clinical judgment and follow-up of an internist will be very important. Involving a hematologist has sometimes helped, but most of them have a narrow interest in oncological cases, so they are often not interested or helpful.

kgoldade Message
29 Mar 2015, 11:15 PM

My 2-and-a-1/2 years' old grandson has SCLS. Do you know if IVIG therapy has worked in someone so young?

Answer

IVIG therapy for children diagnosed with SCLS is a relatively new thing, but as is the case with adults, it is looking very promising. And yes, in a recently published survey of pediatric cases of SCLS, titled "Idiopathic Systemic Capillary Leak Syndrome in Children," which you can see listed at the bottom of the Disorder Resources section of this Community's website, there is discussion of a child (identified as P2) who is 5 years old now and has been on IVIG -- and episode-free -- since she was 33 months old. I suggest that you let your grandson's doctors know about it, and they can then contact the lead author, Dr. Kirk Druey at NIH, with any related questions they may have.

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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.
I´m 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. <p>&nbsp;</p>First acute episode of systemic capillary leak syndrome on May 2003. Failure of different treatments and...
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 t...
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 ...
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 ...
My fiance 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 <p>&nbsp;</p> <p>&nbsp;</p>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 happ...
Mark is my name, I am the partner of my lovely girlfriend Cara, who has recently been diagnosed with SCLS. <p>&nbsp;</p> <p>&nbsp;</p>She has had a difficult time recently, with admissions to ICU ...
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 ...
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 usu...
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 start...
<p>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) an...
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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Discussion Forum

IVIG every 3 months

Created by papa | Last updated 23 May 2017, 06:26 AM

Hemocue

Created by Kimmieroumayah | Last updated 26 May 2017, 03:29 AM

Organ damage

Created by Ritz | Last updated 23 May 2017, 06:16 AM

SCLS Community Member Mahaff

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

SCLS Community Member Mahaff

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


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