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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 ten 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 died in September 2017; Cara O'Hagan (Cara) from Dublin, Ireland, who passed away in February 2018; David Colburn (davec) from Gainesville, Florida, who passed on in September 2019; Jeff Isenhour (jisenhour) from Wahington, DC, who died in September 2021; and Jim Evans (monkeyracing) from Calgary, Canada, who departed this life in August 2023.

 

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 ten 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 died in September 2017; Cara O'Hagan (Cara) from Dublin, Ireland, who passed away in February 2018; David Colburn (davec) from Gainesville, Florida, who passed on in September 2019; Jeff Isenhour (jisenhour) from Wahington, DC, who died in September 2021; and Jim Evans (monkeyracing) from Calgary, Canada, who departed this life in August 2023.

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

Fewer than one in one million people are affected by this disease. The onset of SCLS usually occurs in adults. However, SCLS can affect people of all ages, and our community has patients who were diagnosed with SCLS when they were minors, teenagers, or in their twenties.

Name Abbreviation
Clarkson or Clarkson's Disease Clarkson

Systemic Capillary Leak Syndrome (SCLS) is idiopathic, which means there are no known causes of the condition. Probably a mid-life gene mutation takes place that renders those affected vulnerable to SCLS, or else they are or become immune-deficient in some manner.

Most episodes are triggered by viral infections, including Covid-19, so most 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 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
Vomiting Vomiting
Intestinal cramps Cramps
Diarrhea Diarrhea
Fatigue Fatigue
Headache Headache
Oilguria Sharply decreased or no urine output

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 (abnormally low blood pressure), hemoconcentration (an increase of red blood cells because of a decrease in blood volume caused by the leak of plasma out of the circulatory system), hypoalbuminemia (abnormally low levels of a blood protein called albumin), and the presence of an unusual protein in the blood called a Monoclonal Gammopathy of Unknown Significance (MGUS).

Diagnostic tests may include blood and urine tests to check for abnormalities such as unusually dark urine, hemoconcentrated blood, low serum albumin in the blood, as well as blood pressure readings to check for hypotension.

  • IVIG 1-2 g/kg twice (two infusions) as soon as possible after arrival at the Emergency Room.
  • Methylprednisolone 125 mg intravenously one time, then repeated as needed.
  • Minimal infusion of intravenous fluids (IVF) in boluses and drips.
  • 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 for createnine phosphokinase (CPK) and lactate.
  • Immediate orthopedics consult and repeated compartment-pressure measurement to spot the need for limb fasciotomies if compartment pressures or CPK are abnormally high. 
  • Venous doppler for deep vein thrombosis (DVT) prevention, and may need full anticoagulation to prevent blood clotting.

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

The goal of any saline and vasopressors administered should NOT be to restore a "normal" blood pressure (or urine flow), but to maintain it at a minimal level sufficient to avoid permanent damage to vital organs. Measurement of central venous or arterial pressure in an ICU setting is often necessary to achieve this delicate balance. When too much fluid is administered, the result is excessive swelling, and the patient will usually require surgical decompression of the limbs. In this emergency 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. Many 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.

Recent clinical experience suggests that administration of IVIG with minimal additional intravenous fluids, close to the start of an episode of SCLS, is a safe way to support patients during their leak phase and is associated with rapid clinical improvement.

As concerns episode prevention, two approaches have been tried: high-dose β-agonists like theophylline and terbutaline, and a prophylaxis with high-dose intravenous immunoglobulin (IVIG) infusions. Since 2005, most SCLS patients have been migrated from the former to the latter in Canada, Europe, the United States and beyond, because IVIG therapy leads to far superior results. IVIG infusions every 2 or 4 weeks (1-2 g/kg) prevent or minimize leak episodes far more effectively than any other therapy, and do not have the adverse side effects that treatment with high doses of β-agonists like theophylline and terbutaline entail.

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. For the most part, this treatment has been abandoned.

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

Name Description
Medical help

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

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

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

Clinical Study Volunteer
Patients who have been diagnosed as having SCLS and who are at least 16 years old are wanted for participation in the only scientific study of the illness taking place anywhere in the world: at the National Institutes of Health in Bethesda, MD, right outside Washington DC. You must have a documented medical history including at least one acute episode of SCLS or else continuous symptoms of periodic hemoconcentration, hypotension and protein leakage. Have your primary doctor contact Ms. Robin Eisch at eischar@niaid.nih.gov, before sending in the requisite letter of referral with your medical history and laboratory studies to the lead clinical investigator, Dr. Kirk Druey, kdruey@niaid.nih.gov, tel. 301-435-8875. Once accepted into the clinical research study, you will be invited to come to NIH and spend between 2 and 4 days there for the purpose of being examined, donating blood and a skin sample, and being subjected to various tests (e.g., clinical digital photography of your blood vessels). Depending on circumstances, you probably will have time off to do sightseeing in the capital area during your stay at NIH.

 

Please see the Disorder Resources section.

End of SCLS Study at NIH Created by jenh
Last updated 8 Mar 2024, 02:46 PM

Posted by kevinl1970
8 Mar 2024, 02:46 PM

I was very sad to hear the news of Dr Druey's retirement.  At least in the sense that we will lose a great resource.  I'm of course happy for him, and he has been a great help for me as well as so many other people.

I finally had my first (and last) in-person visit with Dr. Druey on Feb 29 2024, and I think I may have the dubious distinction of being the last patient seen for the SCLS study.  He told us about his retirement during the visit and we were quite surprised.  But all good things come to an end.  He did refer me to Dr Lyons, as Arturo mentioned. He did say he will still be available for another month or two for email.

Posted by aporzeca
8 Mar 2024, 12:05 AM

Hello everyone! Yesterday I was finally able to speak at length with Dr. Druey, whom I discovered and recruited back in 2008 to take on – very much on a part-time basis, given the rarity of the disease and thus the likely limited returns from the effort – the first serious biomedical laboratory research on SCLS. (How I found him and enticed him is a long story, because it was hard to do, but then I got lucky.)

At the time and since then, he devoted himself mainly to the study of asthma, but the irony is that if you fast-forward to nowadays, his nearly 25 published, co-authored articles on SCLS during 2010-2023 overshadow his contributions to advancing knowledge on asthma. And he has said that much to me: If he’s going to be remembered for any contribution to knowledge in biomedicine, it will be, ironically, because of his pioneering research on SCLS.

Dr. Druey told me that he had decided to retire from NIH, a U.S. government agency that allows for earlier and more lucrative retirement than is usual in the private sector, as is the case for all civil servants. This was a shocking surprise to me, because he is relatively young, healthy, and energetic, but evidently, he wants to do something else somewhere else – though he doesn’t have another job lined up as of yet.

He also said that he had tried hard to find someone else, at NIH or elsewhere, to carry on doing research on SCLS and making use of the treasure of patient blood, skin, and other research materials that he has accumulated during the past 15 years, but that so far, he had not found anyone that was interested.

However, he gave me the uplifting news that he has two more scientific articles coming out soon that will leave useful tips to anyone else in the world who might be interested, and that before stepping down this summer he will have written a third article, requested of him by a leading journal, that will summarize all that is now known about SCLS.

Moreover, he will soon be meeting (virtually, I think) with some European, Japanese and Korean experts on SCLS to talk about establishing an international (voluntary) registry of SCLS patients. That way, anyone who would like to do research on this illness will know where to gather medical histories and obtain samples or recruit patients for studies without having to start from scratch. (At present, there is only such registry in Europe, based in Paris, but it’s not comprehensive.)

As concerns Dr. Jonathan Lyons, last year he left NIH and is now working as an allergist-immunologist in California, at the University of California San Diego Medical Center Hillcrest, see https://doctor.webmd.com/doctor/jonathan-lyons-73de8640-da91-40af-a5c9-4f9961821d3f-overview and https://npidb.org/doctors/allopathic_osteopathic_physicians/allergy-immunology_207k00000x/1588860076.aspx 

While he has not carried out any research on SCLS, he became familiar with SCLS patients that visited NIH and he learned about the illness from Dr. Druey. In sum, Lyons is no replacement for Druey, but if you live in Southern California and want to be seen by him as a patient, he will see you.

However, as a reminder, we are lucky to have an expert on SCLS in the East Coast, and specifically in New York City, as well as in Paris, France, please see the section on “Tips and Suggestions” on the page https://rareshare.org/communities/systemic-capillary-leak-syndrome#community_details

In sum, Dr. Druey’s retirement is unfortunate news for all of us because he not only carried out the top-quality laboratory research on SCLS that nobody else had or was doing: he made himself available to advise physicians and patients from all over the world, not only on how to treat patients with SCLS, but also how to deal with medical insurance denials and other practical issues that doctors and patients faced. In this last connection, I encourage everyone who was helped or touched by him to write and thank him for all that he has done for us during the past 15 years.

Dr. Druey will be sorely missed.

Posted by stedrick
7 Mar 2024, 01:44 PM

Will this physician see diagnosed patients that weren't in the study?

Susan

View Full Thread (3 more posts)
Pediatric Patients Created by RevsMom
Last updated 8 Mar 2024, 02:39 AM

Posted by RevsMom
8 Mar 2024, 02:39 AM

II am looking to connect with other parents of pediatric SCLS patients. Our daughter is currently age 7 and receiving twice weekly SCIG infusions. She was diagnosed in May of 2023, after 5 episodes (all post Covid infection in 11/2021 - first leak was 3/2022). She leaks only during viral illnesses. She did not tolerate IVIG; although it did offer us 5 healthy months with no leaks. Her most recent leak was February 2024, when we were switching with insurance to SCIG. That little gap in treatment and an Influenza B virus caused her to leak and resulted in a hospitalization for immediate IVIG. 
 

We are sad to see Dr. Druey retire as he has been a wonderful contact for us during this time. But we are forever grateful for the research he dedicated his time to. 
 

We are treated at Duke Rheumatology. 

Children with SCLS visiting Dr. Druey Created by Itzy11
Last updated 8 Mar 2024, 02:32 AM

Posted by RevsMom
8 Mar 2024, 02:32 AM

Hi Ladies- are your children still doing Subq therapy? Our daughter (age 7) also did not tolerate IVIG and we have since switched to SCIG. She was just diagnosed in May 2023. She was diagnosed during her 5th leak (coinciding with parainfluenza) and had no episodes while in monthly IVIG July-December 2023. We began process of switching insurance to SCIG in January and she unfortunately came down with Flu B which ended up in her 6th leak and a hospital stay. Would love to connect with other parents and see how your children are doing now. Warmly, Jordan Drake 

Posted by leilasmom31
13 Feb 2016, 02:54 PM

Actually - Dr Druey diagnosed Leila . She was basically undiagnosed , the doctors at children's hospital didn't know what was wrong with her and said t see dr Druey and he could diagnose her . He ruled out other causes , and then diagnosed her .leilas doctors at Childrens national hospital consult with him / consulted with him frequently . He will give you reassurance and make sure your doctors are following the most resent / updated protocol for an acute attack. I recommend seeing him or at least speaking to him to get the most current information on a treatment plan. Leila now gets SCIG which is tiny sub q doses of Ivig that is easier to tolerate . She is doing very well with this treatment. Good luck to you and your sweet boy . Remember , you are not alone . Maybe look into scig ? It's definitely easier on the body for a child. My child didn't handle Ivig well , itmade her too sick . Scig has been a life saver for her .

Posted by kgoldade
13 Feb 2016, 01:31 PM

Hi Leilas mom and Enny, my grandson Levi was diagnosed with SCLS about a year ago ( age 2 and a half) and started IVIG once a month then 6 weeks and now 8 weeks and everything was going fine until this last infusion where he got a terrible reaction and the infusion had to be stopped. We are concerned and unsure about continuing ivig. My question is what did Dr Drury do for Leilas when you visited and was anything changed/altered in her treatment after visiting

View Full Thread (2 more posts)
Is IVIG becoming less effective in preventing episodes? Created by aporzeca
Last updated 24 Feb 2024, 03:14 PM

Posted by catsan
24 Feb 2024, 03:14 PM

(Mrs.) DavidS - thanks so much for the information. I will certainly look into it.

:)
Cathy
ps - it took me a long time to see your post due to the struggle to change my email address. I finally gave up and established a whole new account with rareshare.

Posted by DavidS
5 Feb 2024, 06:06 PM

Cathy, I wonder if you're suffering from long covid. I believe some people get long covid from covid vaccines. 

8 months is way too long to feel exhausted and brain-foggy. And now your sinus infection is putting a severe drain on your immune system again.

My husband has SCLS and has gotten all Covid vaccines and boosters with no issues. 

He had COVID a few months ago, took Paxlovid, and recovered within a few weeks.

IVIG continues to work for him after almost 5 years with no leak episodes.

On the other hand, our son has Myalgic encephalomyelitis, also called chronic fatigue syndrome or ME/CFS.

He got sick 19 years ago after a severe flu that was not diagnosed. He has been steadily declining and is now housebound.

We're thankful he's not bedbound on feeding tubes. 25% of people with his disease are.

We opted for him to not take any COVID vaccines, concerned he might have adverse reactions as he has to other vaccines and medicines.

ME/CFS caused by a virus like his (some cases have other causes) is very similar to long covid, they are both post-viral syndromes.

Extreme fatigue and brain fog are among the many possible symptoms.

A few years ago my son had an issue when the COVID virus we've all been exposed to, joined with the Epstein Barre virus in his system that chronically reactives.

Our local doctor worked with Bruce Patterson MD, a long covid specialist, to treat him with antivirals.

The medicine finally helped, but in his case that meant going from 20% functionality to 30%.

Dr. Patterson's protocol may be more helpful for long covid patients because they've only been sick for a few years at most.

I highly recommend Dr. Patterson to anyone who has long covid. https://www.covidlonghaulers.com/home

Hope this helps!

Posted by ValeriaSpain
3 Feb 2024, 11:54 PM

Hi eveyone!, 
Upon reading your experiences and theories about episodes triggered by both covid vaccine and covid infections, I'd like to share that:
1.- Both of my doctors here in Barcelona (the one that diagnosed me with SCLS after my 2nd severe SCLS episode back in 2019 and the one that conducted the research for another treatment apart from IVIG) think that covid vaccines may be as harmful as a covid infection for SCLS patients.

2.- I am not vaccinated and I did contract covid once which resulted in mild symptoms that resolved without hospitalization just as the ordinary chronic and mild leakage episodes that include swelling and decreased blood pressure with dizziness I struggle with every other week since my 2nd severe episode in 2019.
3.- As many of you know, IVIG did not work for me. For the ones who are new: IVIG did not not work for me as I continued to struggle with recurrent and mild leakage episodes that included swelling and decreased blood pressure with dizziness every week/10 days regardless of the IVIG infusions which I received every other week from May up until November 2019, and because my kidneys presented with signs of chronic failure which resolved when the infusions were ceased.
4.- I started daily treatment with ANAKINRA in mid-December 2023, in the midst of one of the recurrent-mild episodes I usually struggle with, which eventually started to escalate to a more severe one. As planned, we gave Anakinra a try and the leakage stopped completely. My Hematocrit levels decreased within hours, my blood pressure normalized within hours too and the 7lbs-oedema resolved within 2 days. I now inject it every morning since that day. 
*Anakinra sold under the name of Kineret, consists of 100mg subcutaneous injections of a prescription medicine called an interleukin-1 receptor antagonist (IL-1Ra) used to reduce the signs and symptoms and slow the damage of moderate to severe active rheumatoid arthritis (RA) in people aged 18 years and older when 1 or more other drugs for RA have not worked, also to treat people with a form of Cryopyrin-Associated Periodic Syndromes (CAPS) called Neonatal-Onset Multisystem Inflammatory Disease (NOMID) and to treat people with Deficiency of Interleukin-1 Receptor Antagonist (DIRA).
5.-I continue taking terbutaline, theophylline, furosemide and bilastine daily.
 

View Full Thread (23 more posts)
An SCLS therapy different from IVIG in the future? Created by aporzeca
Last updated 1 Feb 2024, 02:40 PM

Posted by catsanmice
1 Feb 2024, 02:40 PM

Thank you so much for sharing this hopeful news! I feel like Dr. Druey is an absolute hero for continuing to persue understanding of and treatment for SCLS. This makes me proud that he is originally from Northern California, as am I :)
:D
Cathy

Posted by jenh
21 Jan 2024, 06:43 PM

This is great news. I'm especially happy to think that one of the 18 vials of blood I gave Dr. Druey a few years ago contributed to this study!

Do we know how 4E2 is administered? Infusion, injection, other? I read the free article (skipping the most technical sections) but couldn't figure it out. Thanks!

Posted by acderusha
19 Jan 2024, 02:36 PM

This is amazing news.  Thanks so much for sharing and breaking it down :-).  This community with the leadership of Dr. Druey keep pushing forward to find effective treatments to help those who suffer lead longer fuller lives.  So thankful for all the efforts and dedication these medical experts have put in.

 

View Full Thread (1 more posts)
Immunologist not wanting to follow normal protocol Created by jweb84@me.com
Last updated 1 Feb 2024, 02:32 PM

Posted by catsanmice
1 Feb 2024, 02:32 PM

I started with an IViGg dose of 2g/kg every 4 weeks in 2014. After 1 year, it was decided to put me on 1g/kg. I went along with it because (I think) IViGg can strain the body in some ways, for instance it can be hard on the kidneys, and I have had mild to moderate reactions to it. While my prescription said "every 4 weeks", I usually scheduled myself every 5 weeks. I had no problems with leaks until 2018 when I went 6 weeks between doses - that was a bad idea that landed me in ER.
So 1g/kg of IViGg worked well for me all the way up to Feb. 2021, when I got the COVID vaccines....that resulted in chronic lethergy and mild edema that lasted 8 months, when I finally thought to ask that my IViGg dose be raised a little because I had gained 20 lbs in Jan. 2020 and hadn't had my dosage increased to match my weight. The increased dose made a big difference.
My conclusions from this: 1) I believe 1g/kg is a good dose, generally speaking. 2)  It's improtant to get the doses on the schedule they set you on. 3) COVID and it's vaccines are a stressor for me.
wishing you the blessings of peace and hope - as these are good things that strengthen the immune system :)
Cathy

Posted by aporzeca
31 Jan 2024, 05:10 PM

Please note that Hans had his extra IVIG given by a physician in a hospital.

In the United States, and I presume in most countries, medical insurance companies don't usually second-guess the decisions made by physicians in a hospital setting, so whatever you get in a hospital is not subject to pre-authorizations.

Therefore, should any SCLS patient ever need any extra IVIG in a hurry, the way to get it is to call your main physician, explain the situation, and have him/her make arrangements for you to get the IVIG in the nearest hospital Emergency Room.

Posted by kevinl1970
30 Jan 2024, 06:36 PM

Hans,

I hate to say, in the US, getting a precautionary extra double dose probably would have resulted in a $65,000 bill being sent directly to the patient. Or a pre-approval for something like that most likely would have needed an extra appeal or two, and taken 3+ weeks under emergency circumstances. 

View Full Thread (4 more posts)
Testing Created by anagai2
Last updated 19 Jan 2024, 01:51 AM

Weakness several days after ivig Created by Rita Wood
Last updated 12 Nov 2023, 07:54 PM

IVIG Side Effects Created by rnuara
Last updated 9 Oct 2023, 08:04 PM

Posted by rnuara
9 Oct 2023, 08:04 PM

Just wondering if anyone else who is taking GammaGard has encountered blurry vision after an infusion?  I seem to get blurry vision pretty often after my treatment.

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 External News Link
Title Date Link
What to Know About Capillary Leak Syndrome 09/10/2022
Community Resources
Title Description Date Link
A Tie2-activating antibody reduces vascular leakage in SCLS

A ligand-independent Tie2-activating antibody reduces vascular leakage in models of Clarkson disease

Abstract: We evaluated a monoclonal antibody (4E2) specific for the endothelial receptor tyrosine kinase Tie2 in SCLS patient-derived endothelial cells and reduced baseline and proinflammatory mediator-induced barrier dysfunction. 4E2 also reduced mortality and/or vascular leakage associated with systemic histamine challenge or influenza infection in the SJL/J mouse model of SCLS. These findings support a critical role for Tie2 dysregulation in SCLS and highlight a viable therapeutic approach to this catastrophic disorder.

01/18/2024
Management of Acute Episodes of SCLS with IVIG

Management of Acute Episodes of Clarkson Disease (Monoclonal Gammopathy-Associated Systemic Capillary Leak Syndrome) With Intravenous Immunoglobulins

Abstract: Monoclonal gammopathy-associated idiopathic systemic capillary leak syndrome (ISCLS, or Clarkson disease) is a rare disorder defined by transient but recurrent bouts of hypotensive shock and anasarca resulting from plasma extravasation. Although prophylactic treatment with high-dose intravenous immunoglobulins (IVIG, 1–2 g/kg/mo) prevents most disease flares, its usefulness for acute episodes of ISCLS is unclear. Here the authors report the results of a retrospective study of subjects with acute ISCLS treated at or near the onset of symptoms with IVIG -- six U.S. and European patients during nine attacks. They found that the administration of IVIG with minimal additional intravenous fluids was safe and associated with rapid clinical improvement. IVIG given close to the onset of ISCLS-related symptoms is thus associated with favorable outcomes.

08/10/2022
IVIG Tapering and Withdrawal in SCLS

Intravenous Immunoglobulins Tapering and Withdrawal in SCLS

Abstract: The authors conducted a retrospective, multicenter (more than 50 hospitals in Europe) study including all adult SCLS patients with an MGUS who received at least one course of IVIG, so that made up a universe of 59 patients of mean age 51 (±13 years) followed during the January 1997 to January 2022 period. The overall cumulative probabilities of 2-, 5-, 10- and 15-years survival were 100%, 85%, 72%, 44%, respectively.  IVIG was withdrawn at least once in 18 (31%) patients (W+ group) and never in 41 (69%, W- group). The cumulative probabilities of 10-years survival in the W+ vs. W- groups were 50% and 83%, respectively. The episode relapse rate and the median number of relapses in the W+ vs. W- groups were 72% vs 58% and 2.5 vs 1, respectively.  IVIG tapering was not statistically associated with increased person-year incidence of attacks using a mixed linear model.  IVIG withdrawal, on the other hand, was associated with increased mortality and a higher rate of recurrence in SCLS patients.

08/10/2022
The Consequences of the Covid-19 Pandemic on SCLS Patients

The Consequences of the Covid-19 Pandemic on SCLS Patients

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

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

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

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

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

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

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

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

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

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

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

Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated SCLS

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

01/07/2022
Whole Exome Sequencing of SCLS Patients

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

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

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

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

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

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

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

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

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

The Clinical Picture of Severe SCLS Episodes Requiring ICU Admission

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

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

Capillary leak syndrome: etiologies, pathophysiology, and management

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

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

Idiopathic systemic capillary leak syndrome (Clarkson disease)

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

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

Sharing the Pain [of living with SCLS]

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

 

03/23/2017
The Mayo Clinic Experience with SCLS

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

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

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

Genome-Wide SNP Analysis of SCLS.

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

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

IVIG in SCLS: Report and Review of Literature.

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

03/23/2017
Mechanistic Classification of SCLS

Mechanistic Classification of SCLS.

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

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

High-Dose IVIG Therapy for SCLS.

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

03/23/2017
SCLS in Children

Idiopathic Systemic Capillary Leak Syndrome in Children

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

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

Systemic capillary leak syndrome: recognition prevents morbidity and mortality.

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

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

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

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

03/23/2017
Successful Treatment of SCLS with IVIG

Successful Treatment of Systemic Capillary Leak Syndrome with Intravenous Immunoglobulins.

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

03/23/2017
Comment on SCLS

Comment on The Systemic Capillary Leak Syndrome.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

03/23/2017
IVIG as Treatment for SCLS

Immunoglobulins for Treatment of Systemic Capillary Leak Syndrome

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

03/23/2017
IVIG as Treatment for SCLS

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

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

03/23/2017
The Systemic Capillary Leak Syndrome

Narrative review: the systemic capillary leak syndrome

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

03/23/2017

Clinical Trials


Cords registry

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

Enrolling is easy.

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

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

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

Visit sanfordresearch.org/CoRDS to enroll.

Community Leaders

aporzeca

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

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

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

Given the increased frequency of my episodes of SCLS, despite having taken the recommended doses of the traditional medications (e.g., Theophylline, Terbutaline and Singulair), I was given my first infusion of IVIG in November 2009 and have had monthly infusions since then with no adverse side effects whatsoever. So far, so very good: for over a decade I had no more episodes of SCLS, though lately i had one in January 2020 after coming down with Influenza A days before I was due for my next IVIG infusion; another in December 2021 after coming down with Covid, also shortly before I was due for my next infusion cycle; and yet a third one in April 2023 after being infected with the Human Metapneumovirus, but this time a mere week following my infusion.

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, or only had them during the Covid-19 pandemic.

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, see the Disorder Resources section of our site.  The evidence that IVIG is the best and almost always successful therapy for the prevention of episodes of SCLS is now overwhelming, and now there is emerging evidence that IVIG should also be administered as soon as possible after the onset of an episode of SCLS.

My personal email address is aporzeca@american.edu

 

Expert Questions

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15 Nov 2023, 07:32 PM

Hallo,

Ich bin weibl.56 ,leide an verschiedenen Symptomen die meine Ärzte noch nicht einordnen können.

Seit Nov.2022 ist mein Krankengymnast aufgefallen, dass ich ganz gelbe Handflächen habe.

Ein Bluttest hat keine Veränderung von Bilirubin festgestellt.

 

Ich habe das Gefühl, als könnte ich mit Faust auf und zu machen Flüssigkeit im Gewebe in den Händen rausquetschen.

Ich teile einige andere Symptome wie Durchfall, Ohnmacht, Hitze und Kälteunverträglichkeit und leichte Schwellungen am ganzen Körper. Atemnot bei kleinsten Anstrengungen und bei Hitze. 

Haben andere Mitglieder auch ganz gelbe Hände ? Fussflächen auch?

Grüße vom Bodensee 

Carmen Daum

 

Answer

 

Es tut mir leid, aber nein, gelbe Hände oder Füße sind kein Symptom unserer Krankheit.

wangtai Message
13 Sep 2021, 02:35 AM

Dear Arturo,

https://www.nature.com/articles/s41598-021-93156-1?utm_source=xmol&utm_medium=affiliate&utm_content=meta&utm_campaign=DDCN_1_GL01_metadata_scirep

  Do you think this Canagliflozin novel therapeutic perspectives for this drug in SCLS ?

Answer

This question has not yet been answered

Caroline uk Message
1 Mar 2019, 10:27 PM

Hello Arturo, 

I'm 44 and live in the UK and have been diagnosed a few weeks ago with probable SCLS from my hospital doctors at the end of a 5 day hospital admission with hypotension. I had a previous admission to hospital at the end of September last year with severe hypotension though on that occasion I was in ICU for a week requiring life support which at the time was considered a septic shock reaction though no infection was found. Now I have had these two presentations in hospital fitting the diagnostic triad hypotension, haemoconcentration and hypoalbuminaemia it is considered consistent with SCLS. To treat this I have been given Terbutaline and Theophylline which I am taking. I can see from your and others experience that IVIG is a preferred treatment however. Do you know of any doctors in the UK who have experience of SCLS and treating with IVIG as it is not currently the first line of treatment for this condition here as I understand it. Also from your knowledge is SCLS usually treated under haematology or immunology clinics? 

Looking forward to hearing from you. Kind regards, Caroline.

Answer

A diagnosis of SCLS is not the death sentence it used to be when I got the news 13 years ago.  We've come a long way, and our survival rates are now high, on average.  The operative word is "on average," because according to one of the most comprehensive studies available, "5- 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." (See https://www.amjmed.com/article/S0002-9343(17)30602-2/fulltext)


There are 2 reasons why some doctors, especially those working for underfunded government health plans, hesitate and delay in putting their SCLS patients on an IVIG therapy.  One is sheer ignorance, so in order to combat that, I will email you 4 key, recent articles for you to give to your doctor(s), so they may be informed.  The other is rationing, because since IVIG is extremely expensive while terbutaline and theophylline (T&T) are extremely inexpensive, some physicians know that they won't get approval for an IVIG therapy before first having demonstrated that T&T have failed.  (We have this problem in the USA with the government program called Medicare, which provides health care for the elderly and disabled -- although we're doing something about it.)  The downside risk, of course, is that you never know when the next episode will be your last -- because you didn't survive it.
To expedite a favorable resolution, you can ask and insist that your doctor(s) should obtain a 2nd opinion in writing -- which can then be added to your file to buttress your case -- from one of the two world authorities on SCLS: Dr. Zahir Amoura in Paris or Dr. Kirk Druey in Washington DC, see their contact information on the page "Community Details," under "Tips or Suggestions," see https://rareshare.org/communities/systemic-capillary-leak-syndrome#community_details Your doctor(s) should send in and give you a copy of your hospitalization records, and if you'd like you can also make an appointment to see Dr. Amoura in person if you're willing to visit Paris, see https://www.aphp.fr/offre-de-soin/consultation-37642


And finally, to your last question, the best physician to have on your side is a veteran GP or Internist with a teaching and/or research appointment in a medical school -- namely, someone who is competent AND compassionate.  And since you live in a city with a medical school nearby and a very fine hospital, you should be able to find someone to coordinate your care, and to call on a hematologist or immunologist when needed.  (The latter are familiar with IVIG, the former are less useful.)

Gwendolyn20 Message
4 Jan 2017, 08:33 PM

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

Answer

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

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

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

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

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

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

Rubeanie Message
29 Dec 2016, 04:15 AM

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

Answer

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

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

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

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

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

cpollet Message
20 Nov 2016, 10:27 PM

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

...

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

Answer

Dear Dr. Pollet:

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

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

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

LorraineKH Message
24 Jun 2016, 05:02 PM

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

Answer

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

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

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

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

davec Message
21 Jan 2016, 07:38 PM

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

Answer

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

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

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

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

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

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

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

janiegrrrlaz Message
9 Dec 2015, 07:04 PM

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

Answer

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

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

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

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

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

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

mweston Message
25 Jun 2015, 03:13 AM

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

Answer

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

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My Brother Guy Allen Overland has been diagnosed with Capillary leak syndrome since 2003

Hello

i live in germany and my daughter is 53 years old. she has had an scls crisis every 3 years since 2009.

unfortunately, the doctor who was treating her did not recognize the connection between...

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...

Hospital executive and healthcare attorney/in house counsel. Early retirement due to persistent fatigue and flares.

I was diagnosed with SCLS at Mayo Clinic Jacksonville and have been receiving...

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...

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

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

50 Years old

 

Male

 

Living near London in the UK

 

Married with 3 children (all boys)

 

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

...

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

 

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

 

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

 

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

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

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

 

 

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

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

I have been diagnosed since April 2011

 

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

 

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

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

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

 

 

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

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

wife has capillary leak syndrome

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

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

 

 

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

 

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

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

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

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

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

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

 

 

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

 

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

 

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

My husband's first episode:

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

 

 

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

 

 

Bonjour,

 

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

 

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

 

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

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

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

 

 

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

...

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

 

And yes, they are all ours,

 

no, we have no twins, and

 

no, we are not Catholic.

 

These are always the first...

[Updated on January 2013]

 

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

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

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

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

 

 

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

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

 

 

I have had CLS since February, 2005.

 

 

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

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

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

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

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

 

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

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

Friends, family and patients with SCLS

 

 

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

 

 

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

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

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

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Discussion Forum

End of SCLS Study at NIH

Created by jenh | Last updated 8 Mar 2024, 02:46 PM

Pediatric Patients

Created by RevsMom | Last updated 8 Mar 2024, 02:39 AM

Children with SCLS visiting Dr. Druey

Created by Itzy11 | Last updated 8 Mar 2024, 02:32 AM

Is IVIG becoming less effective in preventing episodes?

Created by aporzeca | Last updated 24 Feb 2024, 03:14 PM

Another Near Fatal SCLS Attack

Created by | Last updated 4 Feb 2024, 01:01 AM

An SCLS therapy different from IVIG in the future?

Created by aporzeca | Last updated 1 Feb 2024, 02:40 PM

Immunologist not wanting to follow normal protocol

Created by jweb84@me.com | Last updated 1 Feb 2024, 02:32 PM

Testing

Created by anagai2 | Last updated 19 Jan 2024, 01:51 AM

Weakness several days after ivig

Created by Rita Wood | Last updated 12 Nov 2023, 07:54 PM

IVIG Side Effects

Created by rnuara | Last updated 9 Oct 2023, 08:04 PM


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