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

 

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

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

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

Name Abbreviation
Clarkson or Clarkson's Disease Clarkson

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

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

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

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

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

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

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 the emergency room (ER) and intensive care unit (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 intravenous immunoglobulin (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.

COVID and Multisystem Inflammatory Syndrome - similarities to SCLS! Created by CaroleTT1222
Last updated 31 May 2020, 11:06 AM

Posted by Annmarie
31 May 2020, 11:06 AM

I am also interested in the role of cytokines in SCLS. 

I suffer from a probable milder but chronic version of SCLS. I have an episode approximately twice a week, although some things influence it like weather where it is worse in rising temperature and air pressue. I have elevated cytokines and my intergrative doctor feel that this is the route of my issue. He says that mast cells are releasing excessive cytokines along with other mediators.

For example, see especially Interlukins 8, 10 and 13

Proinflammatory Cytokines

Interlukin 1    Range  (0.0-2.8)  result  5.9

Interlukin 6                 (0.0-11).            23.6

Interlukin 7                 (0.0-17).            20.7

Interlukin 8                 (0.0-28).           223.2  

Interlukin 17.               < 13.                25.6

TNFa                          (0.0-13)            16.7

TNFb.                         (0.0-156).         106.5

Antiinflamatory Cytokines

Interlukin 4                (0.0-190).          71.3

Interlukin 5                (0.0-13).            55.5

Interlukin 10              (0.0-11)             199.1  

Interlukin 13              (0.0-6)               301.1 

INFg                         (0.0-28)              47.8

Is this a mild cytokine storm ? I am not really sure, but would love to hear the thoughts of anyone else that also ponders these isues. I also worry what Covid-19 might do to me when cytokines are elevated on a good day, although I do feel blessed to live in Sydney where things are currently very mild. 

I hope you all stay well in these difficult times.

Posted by aporzeca
16 May 2020, 02:43 PM

Please take a look at my recent post, https://rareshare.org/topics/1993

Posted by Lolaudesi
15 May 2020, 11:25 PM

Hello, if you have something similar, I attach the link to a medical article that talks about the cytokine storm.  I hope it can be opened because I couldn't paste it.

https://pubmed.ncbi.nlm.nih.gov/32192578/

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Covid-19, SCLS and IVIG: What We Are Learning Created by aporzeca
Last updated 24 May 2020, 11:55 AM

Posted by Barney
24 May 2020, 11:55 AM

Thanks Arturo, I saw this article after reading some of this info and thought it was confirming much of it and maybe adds to discussion:

https://apple.news/ANKHO2xjBQnOvdc_hcOw-qw

 

 

Posted by WazzaACT
23 May 2020, 02:01 AM

Thanks so much Arturo. I have passed to mys specialist who, as always, is very grateful for your insights. I hope you are well.

Posted by DavidS
21 May 2020, 01:09 PM

Thank you so much Arturo for this information, Rebekah Sellers

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Rash/Reaction to IVIG treatment? Created by kmiclon
Last updated 23 May 2020, 10:44 PM

Posted by clswalt
23 May 2020, 10:44 PM

Hello Arturo

I have been fighting a vertabrae problem for about 5 years and have not had any new problems with CLS. Except when I saw this post, I thought I have something to add. In the last couple years, I have had a side effect of hives and swollen face and lips during my IVIG infusion. Most times on the second day. It is rather severe at some times but nothing life threatening. My doctor and Dr. Druey suggested 40 mg of predisone and a 25 mg benedryl two hours before each infusion. This has gone on for about two years. It doesn't happen everytime. I receive an infusion every three weeks. I now find that Benedryl alone can prevent the side effect. I take two 25mg Benedryl right when the IVIG is started. If I experience any itching during, I take another Benedryl. This seems to work the best for me. Like any other side effect, I think we all respond different. You should work with your Doctor on what exactly you should try.

Since I have been taking the Benedryl, I have had very few incidents. The swelling of my face and lips always scare my nurse when it happens, but you just have to monitor yourself. The extra Benedryl usually works in an hour and the itching and swelling subsides. The itching is severe and can drive you crazy, but again not life threatening. I was told no to use an EPI pen unless my airways are blocked. I receive my infusion at home, so there is only the nurse available at the time.

Otherwise, life has been good due to the IVIG. Small problems like this I can take. I hope everyone stays on IVIG. The results have been fantastic. Normal life here except for the Covid 19.

My Doctor and Dr Dreuy suggested our lock-down in late February due to Covid 19. We have done well but we miss our children , friends and neighbors. But the chance of getting the Covid is worth the lock-down. Not sure yet when my Doctor thinks it is ok to come out.

Hope you are well. To Arturo, thank you again for keeping Rareshare going. I appreciate all that you have done for us.

Posted by aporzeca
21 Apr 2020, 11:40 AM

The topic of side effects from IVIG infusions has been discussed several times before, so check out https://rareshare.org/topics/1964, https://rareshare.org/topics/1341, and https://rareshare.org/topics/1282, for example. 

It is possible to have an allergic reaction to a particular infusion or bottle, which is why it is usually given with Benadryl or some such antihistamine, but if so it tends to manifest immediately.  A delayed reaction such as you describe, four days post-infusion, probably has little or nothing to do with the IVIG infusion itself.

You are one of the longest-time members of this community, so please take this opportunity to update your profile page.  It looks like it hasn't been updated since 2009, and it would be good to read how well (or not) you managed during the past decade -- and especially in recent months, ever since you decided, I believe, to receive IVIG on a regular basis.

Posted by kmiclon
20 Apr 2020, 02:23 PM

I am wondering if anyone has had a basic allergic reaction to IVIG treatment,and does this just work itself out?  I had treatment over 2 days, 4/9 & 4/10, seemed to be having easy recovery, but on 4/14 noticed gums sensitive to brushing teeth, then woke up 4/18 & 19 with very sore mouth (like I'd burned it all), and broke out in all-over rash later that day, not itching, just blotchy. Since it was Sunday, went to ER at end of day, had bloodwork and all looked fine, mostly inconclusive, but assuming was combo of IVIG, and sun exposure on 4th day of symptoms (?).  Started itching in the night and now having diarrhea, too.  I have a video-chat with Dr this afternoon, but I am looking for input from others on their experiences, as there is still so much doctors (and I) do not know.

Question to the group -  my understanding of IVIG is that all the treatments/donors are different, so probably the reactions are all different - is that accurate?

Appreciate any input, and so grateful for this resource and all of you - hope everyone is doing well :-)

Albumin Protocol (when admitted to ED) Created by jeffz
Last updated 20 Apr 2020, 06:28 AM

Posted by Windows
20 Apr 2020, 06:28 AM

Jeff could you please email me a copy also  sarah-foreman@hotmail.com

 

I have the original protocol printed out but will add this. Thanks so much

Posted by Shannoncourt
31 Mar 2020, 05:02 AM

Jeff will you email a copy to me please

 

Thanks shannon. shannoncdowell@gmail.com 

Posted by DavidS
30 Mar 2020, 02:57 PM

Makes sense, thanks again Jeff smiley

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Subcu-IVIG? Created by lisamccoleman
Last updated 20 Apr 2020, 04:08 AM

Posted by aporzeca
20 Apr 2020, 04:08 AM

For more answers, check out https://rareshare.org/topics/1939 and https://rareshare.org/topics/1462

Posted by leilasmom31
19 Apr 2020, 05:41 AM

My daughter gets SubQ igg . Hizentra is the brand and she tolerates it much better than the larger infusions . We do the infusions at home 3times a week and it takes around an hour each time . I do it myself and it's very easy to do . Supplies are mailed right to us . She still has side effects but they're mild compared to the large infusions . She has been on Hizentra since around January 2013 I believe and has only had very small leaks a couple times that we just monitored at home . 
Hope this helps . 
Kristy 

Posted by lisamccoleman
18 Apr 2020, 07:09 PM

Hello all... In light of Coved-19 and my on-going side effects from treatment, my specialist and I have been discussing the subcu-IVIG option for me.  I am wondering if anyone has any experience with it or any insight to share with me as we look into this an option going forward. I hope everyone is staying healthy and safe during this time.

Thanks,

Lisa from Canada

First episode of SCLS after 10 years on IVIG Created by aporzeca
Last updated 27 Mar 2020, 11:15 PM

Posted by qnahmed
27 Mar 2020, 11:15 PM

Hi Arturo!  I am sorry to hear about another episode but am so glad to see that you are ok.  It sure makes me nervous to think this even with the ivig therapy, this is a possibility.  Qamer has not had any episodes since 6 years & we have let our guard down.  I will let him know of this news.  Our routine is usually to travel right after getting ivig to reduce the risk.  Qamer is working at a hospital that has 8 corona virus patients & it makes me nervous.  He was able to opt out of caring for them because of the capillary leak syndrome.  You all take care & I pray that everyone stays safe.  

Posted by DavidS
24 Mar 2020, 02:50 PM

Thanks Jeff! If you could scan the document we'd appreciate it. We'll speak to my husband's hemotologist about this as well.

Posted by jeffz
21 Mar 2020, 02:42 PM

Hi Arturo and others -

Haven't been on here for a while and I'm so sorry to hear that you experienced an episode - very glad that you were able to recover!

I'd like to stress again that I have only been on IVIG for about 2 years; for the previous 18 if I had an episode then I followed an albumin infusion protocol when going to the ED. I have used this protocol at least 25 times and it WORKS to abate the leak until it reverses itself. I want to stress this - it immediately makes me feel better and I spend a night in the hospital to be monitored. Then I am home with no side effects other than some IV scabs. I am more than happy to scan the document and send it to the group. 
I did meet with Dr. Druey last summer and have entered into the study - he and his team have this same protocol. 
 

Please let me know if there is a place on here upload the document and I'll be happy to do it.

Good Health to all during this pandemic!

- Jeff Zielinski

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Coping with the Coronavirus threat Created by aporzeca
Last updated 21 Mar 2020, 12:57 PM

Posted by Arielbatt
21 Mar 2020, 12:57 PM

Hello everyone, we have all been stationed since Monday night, where I made all the arrangements to be able to stay 30 days at home without going crazy due to lack of tools.  My wife, a doctor, makes teleconsultations and sells only on Fridays for patients who need a checkup.  I have a huge park that allows me to avoid confinement.  My economy depends on commerce, for what I must handle is stress of knowing how to pay without activity and for how long.  I live in a poor country, so any government help is unthinkable.  I don't see anyone other than my children and my wife, I try to think that this is an unexpected war, where the only important thing is to be alive when the war ends.

Posted by elganzory
20 Mar 2020, 04:39 PM

Hi everybody 

as you wrote before in our experience her in Italy  this Coronavirus is very fast to expand at 15/02/2020 we had 3 persons now we hav right now 41000 persons also in the rest of Europe so i

i recommend to stell at home this is the only way to be safety for you and the other 

i stell ok every thing is going well I get my ivig dose at Monday and Tuesday 

hoping the best for all of us 

God bless us 

Posted by WazzaACT
20 Mar 2020, 05:36 AM

Hi All. May wife and I have self isolated. We are extremely lucky in Australia as we have an very good universal health care system which removes many of the worries that people from other countries can face. My wife and I are also very fortunate to have a holiday house in the Snowy Mountains which are located about 6 hours drive south of Sydney and 2 hours south of Canberra. We have isolated from all friends and family which is already very hard to contemplate but compared to facing a world war for example  is trivial.

Internet, facebook etc makes things so much easier. My wife, an retired early childhood educator, is already assisting one or our family groups with morning book readings along with the other grandparents on alternative days.

I have tried to attach a photo taken from our deck but I dont appear to have the IT skills as yet.

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Can Corona trigger a new episode? Created by HansDeWit
Last updated 10 Mar 2020, 05:09 PM

Posted by elganzory
10 Mar 2020, 05:09 PM

Hi 

I hope that staying well 

as you know I leave in Italy since the virus start I was always at my home I just went to work at the evening to serve the deniers I work like a chef but with the new order we closed the ristorante I am still at home the was the recommendation of the president we hope that this epidemy stop this ovulation. God bless us 

Posted by markc
10 Mar 2020, 11:38 AM

I started working from home last week. My wife has back surgery this week, and the doctors told me that I shouldn't come to the hospital with her. This is a difficult idea to accept, but she is insisting that I stay home.

Posted by aporzeca
10 Mar 2020, 10:53 AM

As Claude wrote, it would appear that all viral infections have the potential to trigger the chain of biochemical events that ends up causing an episode of capillary leak.  Having recently experienced a leak episode while fighting a case of Influenza A infection, see https://rareshare.org/topics/1967, and given the recent spread of the Corona-virus in the United States, my wife and I decided to isolate ourselves at home and give up on seeing friends and our children and grandchildren for a few weeks -- because we don't want to catch anything that they might catch.  Fortunately, I can work from home and "telecommute," and we can Skype or FaceTime or call friends and family.  Isolation is a socially and emotional painful thing to do, because we normally spend time with our grandchildren two or three times a week, and otherwise lead a social life, but we have been urged by our (grown) children to take the threat of potentially deadly contagion very seriously. They would rather not see us for a few weeks but know that we are safe, than risk not seeing us anymore. 

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Any Impact on IVIG availability due to Coronaviris Created by WazzaACT
Last updated 6 Mar 2020, 04:26 PM

Posted by Rita Wood
6 Mar 2020, 04:26 PM

Posted by Rita Wood
6 Mar 2020, 04:23 PM

My concern is the impact on production . The clinic I receive treatment at had a shortage before covid. They also had a shortage of regular iv fluid due to  storms in the production area. I hopes we don’t face problems. I kind of feel there are so few of us with Scls that very few people know about our group or for that matter care.

 

 

Posted by aporzeca
5 Mar 2020, 05:24 PM

I did a search on the Internet and didn't find anything about a shortage of IVIG. 

On the supply side, I saw that the Plasma Protein Therapeutics Association (PPTA), an international organization with offices in Asia, Europe and the USA, had issued a statement (on February 17, 2020, as updated) stating that the coronavirus is not a concern for the safety of plasma protein therapies, including IVIG, manufactured by PPTA member companies, in large measure because "donor screening procedures are in place to prevent individuals from donating plasma who show typical disease symptoms (raised temperature/ fever, cough, difficulty breathing) of a coronavirus infection, including COVID-19.  The SARS-CoV-2 is a large sized virus (approximately 120 nm in diameter). The relatively large size and lipid envelope makes it highly susceptible to steps with virus inactivation and removal capacity used during the manufacturing processes, such as solvent-detergent (S/D), low pH incubation, caprylate-, pasteurization- or dry-heat treatments, nanofiltration or fractionation processes and others. The effectiveness of these processes has been demonstrated on other lipid-enveloped model viruses which are quite similar to 2019-nCoV, e.g. human coronavirus 229E and OC43, SARS-CoV, and porcine coronavirus TGEV" see https://www.pptaglobal.org/media-and-information/ppta-statements/1055-2019-novel-coronavirus-2019-ncov-and-plasma-protein-therapies

On the demand side, however, I see that there is a recent (February 24) WHO report on the virus and it makes mention, on page 37, that in the clinical management of severe and critically ill coronavirus patients, the effectiveness of additional investigational treatment options, including IVIG, is an option that physicians may want to try, see https://www.who.int/docs/default-source/coronaviruse/who-china-joint-mission-on-covid-19-final-report.pdf  Obviously, this is a potential concern for the rest of us users.

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United Healthcare Created by emouzon
Last updated 18 Feb 2020, 04:05 PM

Posted by jisenhour
18 Feb 2020, 04:05 PM

Here's a quick update.  I was able to get ivig approved from United pretty easily.  It seemed that my provider, option care, thought that the approval was not enough.  They wanted payment up front for the treatment and I'd be responsible for getting reimbursed.  They though there wasn't enough data supporting the prescription. I had contact with United via a registered nurse who asked one of their medical directors to call option care and explain the diagnosis, and assure option care payment would be made.  Just in case others are faced with similar issues.

Jeff

Posted by markc
26 Jan 2020, 02:52 PM

United Health Care has been paying for my IVIG for the last 8 years. They rejected my first request but no troubles after that.

Posted by jisenhour
26 Jan 2020, 01:23 PM

Thanks Arturo,  I should hear back Monday about how things are going, and if it looks negative I'll shoot you a note.  I appreciate it.

jeff

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Community News Articles
Default article

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

Publication date: 12 Sep 2016

Community: Systemic Capillary Leak Syndrome

Podcast

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


Community Resources
Title Description Date Link
The 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
Handling Shock in SCLS: Less Is More

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

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

07/09/2019
A natural mouse model reveals genetic determinants of SCLS

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

11/18/2019

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: for over a decade I had no more episodes of SCLS, though I did have one in February 2020, which I hope will prove to be the exception to the rule.

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

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

My address is aporzeca@american.edu

 

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

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

Live in Brisbane, Australia. Recently diagnosed with Idopathic Systemic Capillary Leak Syndrome, including reoccurring Rhabdomyolysis and Kidney damage. Leaks vary from 6kg - 12kg, hospital...

Diagnosed SCLS on February 21, 2019 at 61 years old

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

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

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

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

SCLS - Perth, Western Australia, 28 Male

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

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

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

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

...

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

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

Critical care physician

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

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

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

L

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

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

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

arielbatt@yahoo.com

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

 Wife of the Arielbatt patient and Argentine physician

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

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

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

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

Suspected capillary leak syndrome. Primary immune deficiency/IGG-1
I have been diagnosed with this disease for the last 4 years, and although all at present is going well I would like to be able to talk to others who have this.

 

I live in the Uk and would be...

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

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

Dott.ssa Pollet Cristina-Internal medicine doctor-that work in an Day service Internal Medicine Ward in S. Giovannni Battista Hospital of Turin, North-west Italy
student
Hello my name is Andrea, I am 45 years old and from Germany. I recieved the diagnoses of Capillary leak Syndrome in march 2016.

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

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

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

test

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

I am married to Cristiano, my husband was diagnosed with SCLS february 2016. We live in Denmark with our 2 children (8 and 5 years old).

 

• First episode:

 

October...

Mother of a daughter diagnosed with FMF and SCLS

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

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

 

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

 

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

 

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

 ...

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

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

 

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

Hello,

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

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

Daughter has SCLS

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

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

 

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

 

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

 

in france,

 

who has the "Systemic Capillary Leak Syndrome"

 

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

 

 

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

 

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

 

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

 

Living Sydney Australia

 

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

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

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

 

 

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

 

I am a french woman who suffers capillary leak syndrome.

 

Doctors diagnosed two weeks ago .

 

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

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

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

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

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

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

 

We have a...

 

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

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

 

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

 

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

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

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

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

50 Years old

 

Male

 

Living near London in the UK

 

Married with 3 children (all boys)

 

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

...

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

 

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

 

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

 

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

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

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

 

 

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

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

I have been diagnosed since April 2011

 

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

 

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

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

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

 

 

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

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

wife has capillary leak syndrome

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

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

 

 

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

 

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

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

Diagnosed by the Mayo Clinic in Rochester , MN , with SCLS in August 2011. This was my second trip to Mayo. The first trip was in August 2010 and resulted in a 2 week stay and a suspected / working...
I have just been diagnosed with SCLS -8/18/2010 at the Mayo Clinic in Rochester, MN by Dr. P. Greipp. My first episode of this disease was June 2007. I played a round of Golf and had eaten dinner...
Wife of Mahaff

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

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

 

 

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

 

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

 

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

My husband's first episode:

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

 

 

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

 

 

Bonjour,

 

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

 

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

 

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

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

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

 

 

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

...

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

 

And yes, they are all ours,

 

no, we have no twins, and

 

no, we are not Catholic.

 

These are always the first...

[Updated on January 2013]

 

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

My daughter, age 41, has been battling illness for almost 2 years now. It appears that she has systemic capillary leak syndrome, a very rare condition. We are very anxious to learn all we can about...
I noticed swelling in my ankles in spring of 2007, which increased through that summer. I was working full time at a desk job, then on evenings & weekends at our home business helping with...
family physician , patient with scls
My first attact was in Oct 2005. I was disgnosed in June 2006 with Capillary Leak Syndrome. The first time I entered Mass General in Boston Mass the Dr's thought I had a virus due to the fact that...
I was just diagnosed with scls and am looking for any information on it, since my doctor was unable to give me much of anything yesterday. So I am taking it upon myself to do some of the research...
sono la moglie di mario gatto se qualcuno in italia vuole contattarmi per avere informazioni sulla malattia puo' farlo su facebook mi chiamo marcella iannacchero
I live in Christchurch, New Zealand, and if I could find some way of connecting with some supporting people I would be very grateful.

 

 

I have been living with SCLS since October 2005. I...
Name: Walt Breidigan, Bethel Park, Pa. Born 1957

 

 

I have had CLS since February, 2005.

 

 

The first incident gave me two total drop feet and no feeling from calves to the toes...
Heading a research study on Systemic Capillary Leak Syndrome at the National Institutes of Health.

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

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

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

 

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

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

Friends, family and patients with SCLS

 

 

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

 

 

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

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

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

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Discussion Forum

Surgical Procedures?

Created by cmanmommy | Last updated 27 May 2010, 05:12 AM

SCLS Study - NIH

Created by Ritz | Last updated 17 May 2010, 12:24 PM

CLS History of Individuals

Created by clswalt | Last updated 8 May 2010, 05:36 AM

Terbutaline vs. Theophylline

Created by Josephite | Last updated 29 Mar 2010, 06:26 AM

Need help from IVIg patients

Created by Josephite | Last updated 25 Mar 2010, 06:56 PM

Any Ideas?

Created by timpro | Last updated 24 Mar 2010, 03:29 AM

FOUNDATION

Created by cmanmommy | Last updated 19 Mar 2010, 02:08 PM

SCLS in child

Created by sclsusr1 | Last updated 18 Mar 2010, 04:55 PM

Thank you Walt

Created by jordansh | Last updated 17 Mar 2010, 02:00 PM

SCLS on U.S. TV Tomorrow

Created by aporzeca | Last updated 16 Mar 2010, 01:29 PM


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