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 eleven 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; Jim Evans (monkeyracing) from Calgary, Canada, who departed this life in August 2023; and Caroline Hollingsworth (Caroline UK) from Chester, United Kingdom, who passed away in July 2024.
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 eleven 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; Jim Evans (monkeyracing) from Calgary, Canada, who departed this life in August 2023; and Caroline Hollingsworth (Caroline UK) from Chester, United Kingdom, who passed away in July 2024.
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 |
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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 |
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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.
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 | |
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Medical help |
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Clinical Study Volunteer |
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Please see the Disorder Resources section.
What I understand is Dr. Druey's final research contribution to the scientific literature on SCLS has now been published: a comprehensive scholarly article titled simply "Systemic Capillary Leak Syndrome" appearing in the journal Nature Reviews Disease Primers, freely available at https://www.nature.com/articles/s41572-024-00571-5.pdf
The article is written by medical scientists for other medical scientists and for physicians who see patients, and so it's not easy reading for us mere patients or caregivers. However, I recommend that everyone download the article and pass it on to your current and any future physicians you see, because it provides a comprehensive summary of what is known -- and not yet known -- about SCLS.
(In terms of what to always have handy and take with you to the Emergency Room, I still recommend that you take the following article, see https://rareshare.org/topics/2192: https://www.acpjournals.org/doi/pdf/10.7326/aimcc.2022.0496?download=true)
It was back in 2008 that I succeeded in recruiting Dr. Kirk Druey, then a mid-career, physician-scientist doing research on asthma at the prestigious U.S. National Institutes of Health, to carry out original research on SCLS -- something he agreed to do but very much on a part-time basis. Finding him was like looking for a needle in a haystack: it took me almost two years to do so because at the time NIH had more than 1,000 staff scientists, and none of them knew anything about SCLS.
In the end, it was a case of serendipity: at a dinner in Washington DC, my wife was randomly seated next to an NIH staff hematologist-scientist. As we learned later, he was very senior and accomplished, so much so that he would go on to win the 2020 Nobel Prize in Medicine. And fortunately, she took the opportunity to ask him if he might be able to help us find someone within NIH who might be willing and able to do some research on a rare blood disorder called SCLS. Miraculously, he agreed, though he told her that it would take him a number of months -- and so it did.
When I met him, Dr. Druey told me not to expect him to find a cure for SCLS within my expected lifetime, because in those years, before treatment with IVIG became increasingly successful and thus common, patients with SCLS usually died within at most 5 years of their first episode. What he committed to do was to carry out original laboratory research on SCLS to determine, at the very least, the cause of the very unusual illness.
Fast forward to 2024, and we still don't really know why some of us -- maybe one in a million -- have the condition. And while we can avail ourselves of a blood product (IVIG) that helps to prevent most of our episodes and thus to lengthen our expected lives, a cure has yet to be found.
This is so despite Dr. Druey's best efforts, as evidenced by the fact that during the past 16 years, he has managed to co-author more than 25 scientific articles on SCLS, see https://pubmed.ncbi.nlm.nih.gov/?term=(Druey%20KM%5BAuthor%5D)%20AND%20(capillary%20leak%5BTitle%2FAbstract%5D)&sort=pubdate
Indeed, as Dr. Druey himself told me a few years ago, "it is ironic that while I've dedicated myself full-time throughout my long scientific career to medical research on the causes and cures for asthma, I will be remembered for my part-time work on SCLS."
Jordan, congratulations on such a great initiative you have been pursuing!
Dr. Pierce and colleagues have already published 3 research pieces on SCLS, so I'm pretty sure that he knows what he's doing.
The highly technical articles can be viewed at https://rupress.org/jem/article/214/12/3497/42272/A-p190BRhoGAP-mutation-and-prolonged-RhoB (2017), https://journals.lww.com/pccmjournal/abstract/2018/07000/sera_from_children_after_cardiopulmonary_bypass.2.aspx (2018), and https://journals.lww.com/shockjournal/fulltext/2019/08000/whole_exome_sequencing_of_adult_and_pediatric.7.aspx (2019). Dr. Druey was one of Pierce's coauthors in this last one.
Dr. Druey and two co-authors have just published a comprehensive primer on SCLS, both primary (ISCLS -- the kind we in this community have) and secondary (SSCLS), to which I dedicate a separate Topic entry here in our RareShare home, see https://rareshare.org/topics/2260, and they cite Pierce's three aforementioned articles, see https://www.nature.com/articles/s41572-024-00571-5.pdf
In case he hasn't seen it, please mention the publication of this primer to Dr. Pierce, because there is research advice in it for him: "Because preliminary analysis has thus far failed to uncover shared exomic aetiologies in adult patients [Pierce’s 2019 article cited here], further scrutiny of non-coding DNA sequences is warranted. The discovery of autonomous and durable functional defects in MG-CLS-derived endothelial cells points to the possibility of de novo somatic mutations limited to the endothelium. In contrast, de novo germline mutations are more likely in children such as the potentially pathogenic mutations in ARHGAP5."
Hi All! This note is for *pediatric* patients only (including those who are adults now, but were diagnosed as children), although we are sending well wishes and hugs to everyone from North Carolina. Xx
I have been in touch with Dr. Richard (Rick) Pierce from Yale University with exciting news about taking a closer look at genetic sequencing for kiddos with SCLS. (thank you to Whitney Turner Septon for expediting our notes in his inbox).
In sharing Reverie's story with Dr. Pierce I expressed my interest in needing to do a better job at educating major U.S. PICUs and perhaps major children's hospitals around the US about SCLS. While the disease is incredibly rare, the presentation is so simple and identical in every single case (periorbital edema, hypoalbumenia, tachycardia, hypotensive). I believe we have the ability to save another child's life by simply informing major US hospitals about the presentation and symptoms of SCLS. I am aware of 3 pediatric cases that developed following 2021 Covid infections; I imagine there are several others out there right now who are on the same runaround journey we all were with Allergy/ENT, etc.
Well, HE LISTENED. And I received the best email:
"I am working on putting together an awareness campaign and educational module on the diagnosis and inpatient treatment of pediatric SCLS. As part of this campaign, we would love to include patient and family stories. We are working with Yale's Department of Pediatrics media team and Yale's media production teams.
On November 25, we are doing an interview with Yale's Media Team to tell the story of Reverie's diagnosis and treatment.
Dr. Pierce's team also feels there is a genetic component to SCLS and investigated eight pediatric SCLS patients with genetic sequencing through Yale's Pediatric Genomic Discovery Program (manuscript attached). Unfortunately, they did not find any single genetic abnormality that could explain all pediatric cases of SCLS. However, it was only 8 patients and they are always keen to sequence other patients. They also did the analysis in 2018 and have learned more about SCLS; they can re-analyze all of our data to see if something new comes up.
Please let me know if you and your child are willing to participate in the genetic sequencing, the more samples the better. I will put you in touch with the study admin at Yale, Monica. It is a very simple saliva test (no cost to participate)
They are waiting to sequence all samples to reduce confounding batch effects.
With Love,
Jordan, George & Reverie
PS: I do have a PDF copy of the 2018 study if anyone would like to see it.
https://www.yalemedicine.org/departments/pediatric-genomics
My husband had IVIG at home for years paid for by private insurance. In fact, because I am a nurse I was able to administer it myself. But when he went on Medicare he had to go to an infusion center. There is no other way Medicare will pay for it.
Hi, my husband started infusions in 2019 and was never offered at home. He's been on Medicare for 2 years and still gets infusions at the hospital. We didn't know home infusions were an option.
Hello, I have been getting IVIG at home infusions for nearby four years under private insurance. Starting in July of this year I have been on Medicare and only been able to get infusions through an Outpatient Infusion center. I have been unable to get at home infusions set up through Medicare by my Provider. My question is "are any of you located in the USA currently getting in home infusions of IVIG through Medicare?"
Hello!
Back in 2019 when I was still working out (but not aware of the edema being caused by capillary leak), I also took creatine based on recommendations from a personal trainer. I did notice I started to retain a litle (not a lot) more water weight from the creatine powder, but I can imagine this works differently for everyone. I have however stopped taking this supplement, because the added water weight made my lower body feel more tight and uncomfortable. I can imagine that through firstly minor dietary adjustments, you can gradually increase creatine intake and monitor the effects on your body, without getting a very big amount of creatine from a supplement all at once (for reference, there is about 2 grams of creatine in a whole raw chicken). I hope this helps! :)
Hello, I hope you're all felling alright. A wile ago I started to do a training. The coach suggested me to eat 5 grams of creatine every day. I read that the creatine produces a little of intramuscular, not superficial, fluid retention. That scared me a little, to be honest. Do any of you have some experience with this supplement? I would ask my doctor, but I know that if there is even a slightest doubt, we will tell me not to take it.
Beste Hans,
Bedankt voor het berichtje!
Via een andere hele vriendelijke en behulpzame patient op dit forum is inderdaad de ingang tot het AMC tot stand gekomen, waar ik erg dankbaar voor ben. Er is vanuit mijn hematoloog in het Rijnstate een brief aan de voorgestelde arts in het AMC gezonden. Ik ben nog in afwachting van reactie.
Wat heftig van de aanval in Bretagne... Gelukkig heeft u het overleefd! Fijn dat er begeleiding was van Marc Pineton de Chambrun. Ik ben ook dankbaar dat hij de moeite doet om met mijn casus en die van vele anderen mee te denken.
Wat ontzettend fijn om te vernemen dat het nu goed met u gaat! Ik hoop van harte dat u aanvalvrij mag blijven! Ik kan me voorstellen dat het krijgen van Covid wel een spannende situatie was. Bij griep (vaccinatie) heb ik ook vrij heftige aanvallen beleefd. Welke virusremmers heeft u toen gekregen? Gelukkig heeft u Covid in die zin goed doorstaan!
Vriendelijke groetjes,
Marielle
Marielle
1. UMC Amsterdam lijkt me een goed alternatief om de mogelijkheid van SCLS te onderzoeken. Op dit forum zijn nog zeker 2 patienten aanwezig wiens case daar in behandeling is. Kijk maar even rond bij de leden op dit Forum.
2. Marc Pineton de Chambrun van Salpêtrière Parijs heb ik nooit persoonlijk ontmoet maar hij heeft de artsen van het ziekenhuis van Brest (F, Bretagne) begeleid bij mijn opname. In Bretagne heb ik op vakantie in 2019 een zware bijna fatale aanval gehad met coma en fasciotomie aan onderbenen en onderarmen gehad. Salpêtrière Parijs is dan ook het Europese kenniscentrum voor SCLS. Zahir Amoura en Marc Pineton de Chambrun zijn de belangrijkste artsen op dat gebied.
Momenteel gaat het goed met mij, ik ontvang elke 3 weken 1g/kg IVIG en sinds 2019 aanval vrij. We blijven altijd erg attent bij het minste teken van ziekte. Enkele weken geleden heb ik voor eerst Covid gehad maar goed doorstaan zonder al te ziek te zijn maar ik heb wel onmiddellijk virusremmers voorgeschreven gekregen van mijn hematoloog.
Hans
I also recommend Suzanne Hayman (Hematologist Oncologist) at Mayo Clinic in Rochester, MN.
Hi Whitney!
I hate these circumstances, but I am so happy you found this group. Please feel free to text me when you can, 630-903-9318.
We have an amazing 7 year old daughter, Reverie- who is an SCLS warrior. And an incredible team of Rheumatologists at Duke and UNC. Here's a snip of our story below from right after diagnosis in May 2023..
"In April 2022, Reverie woke up one day with swollen eyes. We of course thought she was having an allergic reaction and phoned the pediatrician and followed the OTC course of Zyrtec and Benadryl. The medicine didn’t seem to make the swelling go down at all, but after a few days, it subsided and we thought, ok, freak incident.
Well, just a few weeks after, it happened again. This time I noticed it was when she had a fever during a virus, with cold like symptoms. This prompted my Mom instinct and we knew then this is not allergy related, something else is going on.
Over the past year, Reverie has had 5 episodes of this periorbital edema, anytime she is sick. We have met outpatient with ENT, had sinus imaging done, pediatric ophthalmology. You name it, we checked it.
On the week of May 5th, Reverie once again developed a typical kiddo cold virus and we knew the eye swelling was following close behind. This time she became incredibly ill and lethargic prompting a trip to the nearest emergency room. Her blood work revealed dangerously low sodium and albumin (a protein), and she was admitted immediately.
We spent the next 5 days admitted to Duke Children’s Hospital meeting with Nephrology, Endocrinology, Allergy & Immunology, Gastroenterology. All teams were puzzled as to what was causing the swelling and where it was coming from. Reverie gained 4 pounds of fluid while inpatient, over 10% of her body weight in two days. And by the grace of God, the last team to meet with, Rheumatology, believed they knew what Reverie had.
After much more testing, and several consults Reverie has received an official diagnosis of exclusion of:
Systemic Capillary Leak Syndrome (SCLS) or Clarkson’s Disease."
Talk Soon!
Warmly,
Jordan Drake
Raleigh, NC
630-903-9318
Hello everyone!
I just found this group while doing google searches for SCLS. My 7 year old daughter was diagnosed last week after her third hospital stay. She has had several other mild leaks but I thought they were just allergies since the swelling was mostly located around her eyes and face. We have been referred to the rheumatologist and will see them in a few weeks. We will be doing genetic testing through someone at Yale University as well. I would greatly appreciate any advice or contact you are willing to share.
Thank you,
Whitney
I am having more issues after IVIG, I am a longer term patient. On later the third after treatment I start to feel weakness starting. On the fourth day walking is an issue I can barely walk to the bathroom which is less the 50 feet away. Now my arms are starting to get weak too. I have deep bone pain both the third and four day. By the fifth day I am feeling stronger. Is anyone else having these issues?
Ritz
Dear krogers,
I'm very sorry that you took offense, but to clarify (a) I was not referring to you but, rather, to dozens of patients I have heard from over the past 15+ years, plus the many I have read about such as in the publication I cited; and (b) I actually wrote "Sometimes it is by patient choice or perceived necessity, but my impression is that most often it is the result of the rationing of IVIG..." so I definitely ackowledged those, like you, who freely chose to receive lower doses.
I'm very happy that in your case you've been episode-free for over 15 years. I wish I could say the same, despite having received a much higher dose than you.
Now, if you have factual reasons for implying that rationing of IVIG for SCLS patients in the USA is a greater problem than in the whole of Europe, please let me know because that would be shocking news to me!
I am probably who Arturo is refering to with rationing on government led health care systems.
This is simply inaccurate and I feel inappropriate. Not going for the maximum dose was a patient centred descision which has resulted in better treatment and what is more not a single leak in over 15 ytears.
Rationing of healthcare in the USA is rather a greater problem than in the the whole of Europe.
I have the greatest respect for Arturo but this I felt needed a response
Hi
I had diagnosed at 2010 I had start ivig for the first year 2gr/kg and starting from the second year it was 1gr/kg until now some times I had a problem like Covid 19 under acut attack they give me 2gr/kg just in acut attack
rig now everything is ok
hoping the best for all of you
and special thanks for Arturo
yaser
Thank you for the great suggestions. I stay with the same office for care. The doctor I was temporarily assigned to has already left. Rightnowi am with a nurse practitioner that knows both me and my case. I am a like more comfortable now.
Ritz,
Well, yes, this is a relatively good problem to have. Compared to the situation 2 decades ago, when a diagnosis of SCLS was essentially a death sentence, most SCLS patients are now able to live for decades beyond their first episode – so much so that our trusted and caring physicians are retiring before we die, and when they do we rightly feel abandoned and fear for the continuity of our care. But at least we are alive!!!
I have a few related suggestions. First, let your most knowledgeable physician know you worry about the continuity of your care, and that if and when he/she plans to retire or move on, they will need to give you advance notice so that together you can transition to finding, meeting, and educating another (presumably younger but qualified) physician. (I realize, this piece of advice comes a little late for you.)
Second, let your most knowledgeable physician know that he/she should formally, in a piece of official stationery, provide written instructions for the continuity of your care. These can be entered into your official records and be printed out for you to carry with you always, including on your phone, ready to be shown to the Emergency Room’s triage nurse and on-duty physician. These instructions should include: (a) what tests to run and measurements to take to confirm that you are having an episode of SCLS; and (b) what medications and in what dosage you should be given how often to help you survive your episode without any damage to organs or limbs. (It's probably not too late for you to obtain such instructions.)
Third, carry with you in electronic and/or printed form the one or two articles that best describe your condition and that spell out what to do about it in case of an episode, see for example https://rareshare.org/topics/2192
And fourth, make sure that you never go alone to the hospital. We all need a clear-minded and duly informed relative, friend or neighbor who can advocate for us, help tell or confirm our story, and make sure that nurses and physicians are listening and doing their reading (e.g., of our doctor’s instructions).
Arturo
Hello Rita, you should again look for a general practitioner, a family doctor, who preferably works in a hospital in your city, where they have your medical history and who can contact you whenever you need it. It may not be easy, but your previous doctor could recommend you to a colleague.
For a recent (October 2023), comprehensive article on Urolithin A's effects see "Pharmacological Effects of Urolithin A and Its Role in Muscle Health and Performance: Current Knowledge and Prospects," available at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10609777/
Its final paragraph reads: "In conclusion, key findings regarding Urolithin A in muscle health and performance encompass its regulatory effects on promoting muscle protein synthesis while inhibiting degradation, its interactions with crucial signaling pathways, potential enhancement of endurance and fatigue resistance, as well as its anti-inflammatory properties. These findings establish a solid scientific foundation for the potential application of Urolithin A in improving muscle health, promoting muscle growth, and enhancing exercise performance. However, further research is needed to validate these findings and to explore the safety, optimal usage, and suitable populations for Urolithin A."
Thanks so much Cristina for sharing this information. And welcome back!
Rebekah Sellers
Hello All,
I have been missing from this community for quite some time due to, of all things, technical issues with an email address change. The problem is finally fixed and I am glad to be able join discussions. A lot has happened since the last time I was on this site, and I would like to share some information with you. Ever since the Covid pandemic in 2020 my CLS episodes had been more frequent and more severe. I have contracted Covid four times and have had the vaccination and boosters. My doctor and I were in touch with Dr. Druey many times as we tried to figure out the problem and the solution. We tried changing Ivigg brands as I was having a leak after every infusion, but that didn't help. We changed my infusions from intravenous to subcutaneous and this helped some. But in October of 2023 and in January of 2024 I had two more life threatening attacks. After the October attack, I had to be put on dialysis for several weeks. Luckily my kidneys improved and I was able to get off of dialysis. Becasue of the October attack, the hospital staff knew how to treat me more effectively in January. To avoid kidney failure they immediately placed me on CRRT (continuous renal replacement therapy). It worked. They also used vasopressors and albumin to keep pressure in my veins and keep me hydrated while they administered Ivigg. This combination helped me have a shorter hospital stay and recovery time.
The biggest news is that another doctor, who is a friend of the family, had been taking a Urolithin A supplement. He suggested I try it to see if it would benefit me. I began taking it in late January. It has done wonders. In the first three months I noticed the frequency of leaks beginning to slow down and the leaks were not as severe. I now have not had a Capillary Leak since April. I am back to teaching fitness classes, traveling, living a normal life, and I feel stronger than I have in years. I took the supplement to my doctor who manages my CLS to get his thoughts on Urolithin A. After reading articles and reviewing many studies from the NIH on Urolithin A, he suggested I share this information with the CLS community in hopes that it could help someone else.
I continue to do my subcutaneous Ivigg treatments 2x a week.
Best Regards,
Cristina Burns
Publication date: 12 Sep 2016
Community: Systemic Capillary Leak Syndrome
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)
Title | Description | Date | Link |
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The Systemic Capillary Leak Syndrome |
Narrative review: the systemic capillary leak syndrome Abstract: The systemic capillary leak syndrome (SCLS) is a rare disease of reversible plasma extravasation and vascular collapse accompanied by hemoconcentration and hypoalbuminemia. Its cause is unknown, although it is believed to be a manifestation of transient endothelial dysfunction due to endothelial contraction, apoptosis, injury, or a combination of these. Fewer than 150 cases of SCLS have been reported, but the condition is probably underrecognized because of its nonspecific symptoms and signs and high mortality rate. Patients experience shock and massive edema, often after a nonspecific prodrome of weakness, fatigue, and myalgias, and are at risk for ischemia-induced organ failure, rhabdomyolysis and muscle compartment syndromes, and venous thromboembolism. Shock and edema reverse almost as quickly as they begin, at which time patients are at risk for death from flash pulmonary edema during rapid fluid remobilization. Diagnosis is made clinically and by exclusion of other diseases that cause similar symptoms and signs, most notably sepsis, anaphylaxis, and angioedema. Acute episodes are treated with vasopressor therapy and judicious fluid replacement, possibly with colloid solutions for their osmotic effects, to prevent the sequelae of underperfusion. Prognosis is uncertain, but patients who survive an initial severe SCLS episode are estimated to have a 10-year survival rate greater than 70%. Much remains to be learned about SCLS, and clinicians should consider the diagnosis in patients with unexplained edema, increased hematocrit, and hypotension. |
03/23/2017 | |
IVIG as Treatment for SCLS |
High-Dose Intravenous Immunoglobulins Dramatically Reverse Systemic Capillary Leak Syndrome. Abstract: The objective of this study was to report the dramatic improvement of patients with systemic capillary leak syndrome obtained with high-dose intravenous immunoglobulins. Systemic capillary leak syndrome is a rare and life-threatening disorder characterized by hypotension that can lead to shock, weight gain, hypoalbuminemia, and elevated hematocrit secondary to unexplained episodic capillary fluid extravasation into the interstitial space. Because its cause is unknown, systemic capillary leak syndrome treatment has remained largely supportive. Intravenous immunoglobulins administration to a patient with refractory systemic capillary leak syndrome yielded dramatic improvement. The patient is still alive 11 yrs after systemic capillary leak syndrome diagnosis and receives intravenous immunoglobulins monthly. Later, based on that result, intravenous immunoglobulins were successfully given to two other patients during the acute phase of systemic capillary leak syndrome. Both are still alive 8 and 1.5 yrs after receiving intravenous immunoglobulins at the onset of each flare. In conclusion, intravenous immunoglobulins were effective against systemic capillary leak syndrome symptoms in three patients, but their exact mechanism remains unknown. Their immunomodulatory effect merits further investigation. |
03/23/2017 | |
IVIG as Treatment for SCLS |
Immunoglobulins for Treatment of Systemic Capillary Leak Syndrome Abstract: A 43-year-old white woman in France diagnosed with SCLS was put on the recommended combination of Theophylline plus Terbutaline, but she nevertheless had 10 episodes of severe capillary leak during 2001-mid-2007, necessitating intensive care unit admission for her last 3 episodes. She was then put on IVIG administered every 6 weeks, and this yielded a dramatic improvement such that she has had no more episodes and has returned to her normal lifestyle. |
03/23/2017 | |
Mayo Clinic write-up on SCLS |
The Mayo Clinic's summary of the diagnosis and treatment of SCLS. During an episode of systemic capillary leak syndrome, fluids are administered intravenously to maintain the patient's blood pressure and to prevent damage to vital organs such as the kidneys, heart and brain. The amount of fluid must be carefully controlled. An attempt to normalize blood pressure through aggressive fluid administration can cause destructive swelling of the body's extremities and overload the kidneys and lungs when the body needs to eliminate the excess fluids after the episode passes. Glucocorticoids (steroids) are often injected during an acute capillary leak syndrome attack to reduce or stop the capillary leak. This is sometimes successful. Fluid pressure in muscles may be monitored. Emergency surgery may be needed to relieve pressure and minimize damage to muscles and nerves in the arms and legs. Once the capillary walls stop leaking and fluids start to be reabsorbed, patients are usually given diuretics to speed up elimination of the fluids before they accumulate in the lungs and other vital organs, which can be a fatal complication. Patients who avoid organ and limb damage in a capillary leak syndrome episode tend to recover their health after several days, once the capillary walls return to normal and the accumulated fluid is expelled from the body through urination. Although no cure has been found for systemic capillary leak syndrome, the frequency and/or severity of episodes is often reduced by having patients take certain asthma medications: theophylline and terbutaline. Patients also may benefit from intravenous treatment with immunoglobulin or by taking thalidomide. Patients may also be prescribed corticosteroid pills such as prednisone to be taken at the first sign of symptoms of another capillary leak. |
03/23/2017 | |
Lessons from 28 European Patients with SCLS |
The Systemic Capillary Leak Syndrome: A Case Series of 28 Patients From a European Registry. Abstract: The article describes the clinical characteristics, laboratory findings, treatments, and outcomes of patients with SCLS who were not previously reported in the literature. These European patients with SCLS were treated and monitored from the start of 1997 until end-July 2010. Survival rates were 89% at 1 year and 73% at 5 years; instances of death were directly related to SCLS attacks in 6 cases (75% of total). Treatments of various kinds increased the chances of survival: Five years after diagnosis, survival rates were 85% in 23 patients who had received a treatment and just 20% in 5 patients who had not. The authors provide additional evidence that a prophylactic treatment with IVIG tends to reduce the frequency and severity of attacks, and may improve the survival of patients with SCLS. |
03/23/2017 | |
IVIG: A Promising Approach to SCLS |
High-dose intravenous immunoglobulins: A promising therapeutic approach for idiopathic systemic capillary leak syndrome. Abstract: The article reports the case of a 40-year-old woman with chronic SCLS treated in Berne, Switzerland, with high-dose intravenous immunoglobulins (IVIG) after a prophylactic therapy with theophylline and terbutaline (T&T) was poorly tolerated and failed to decrease the frequency and severity of the attacks. During the 5 years she was on T&T the patient suffered from about 20 similar episodes of mild to moderate shock, often requiring hospital re-admission and supportive therapy. So far, 10 months of prophylactic therapy with IVIG (2gr/kg/month) have resulted in an impressive reduction of intensity and frequency of attacks, confirming the finding of other case studies. |
03/23/2017 | |
Comment on SCLS |
Comment on The Systemic Capillary Leak Syndrome. Abstract: The authors report on 2 additional patients from the United States with SCLS in whom prophylaxis with terbutaline and theophylline failed, but who had no further episodes after the initiation of IVIG therapy. There are additional published reports of successful prophylaxis with IVIG cited, and the authors are also aware of yet another case. Given the present state of knowledge and despite the high cost, the authors strongly believe that IVIG is the optimal prophylaxis and should be the initial choice to prevent attacks in patients with SCLS. |
03/23/2017 | |
Successful Treatment of SCLS with IVIG |
Successful Treatment of Systemic Capillary Leak Syndrome with Intravenous Immunoglobulins. Abstract: The authors report on a 48-year-old woman in Spain who had her 1st episode of SCLS in 1997 and was initially put on a regimen of terbutaline and aminophylline, but went on to endure 20 additional episodes in the subsequent 3 years. She was then treated with melphalan-prednisone for a year and the frequency and intensity of her episodes diminished and even disappeared. In 2005, however, the episodes returned and in 2008 she was finally put on a regimen of IVIG (2 g/kg) every 6 weeks. She has had no more episodes since then. |
03/23/2017 | |
Laboratory Evidence of SCLS and of the Effectiveness of IVIG |
Vascular Endothelial Hyperpermeability Induces The Clinical Symptoms of Clarkson Disease (The Systemic Capillary Leak Syndrome) Abstract: The authors report clinical and molecular findings on 23 subjects, the largest SCLS case series to date. Application of episodic SCLS sera, but neither the purified immunoglobulin fraction nor sera obtained from subjects during remission, to human microvascular endothelial cells caused vascular endothelial cadherin (VE-cadherin) internalization, disruption of inter-endothelial junctions, actin stress fiber formation, and increased permeability in complementary functional assays without inducing endothelial apoptosis. Intravenous immunoglobulin (IVIG), one promising therapy for SCLS, mitigated the permeability effects of episodic sera directly. Consistent with the presence of endogenous, non-immunoglobulin, circulating permeability factor(s) constrained to SCLS episodes, we found that two such proteins, vascular endothelial growth factor (VEGF) and angiopoietin 2 (Ang2), were elevated in episodic SCLS sera but not in remission sera. Antibody-based inhibition of Ang2 counteracted permeability induced by episodic SCLS sera. Comparable experiments with anti-VEGF antibody (bevacizumab) yielded less interpretable results, likely due to endothelial toxicity of VEGF withdrawal. Our results support a model of SCLS pathogenesis in which non-immunoglobulin humoral factors such as VEGF and Ang2 contribute to transient endothelial contraction, suggesting a molecular mechanism for this highly lethal disorder. |
03/23/2017 | |
Systemic capillary leak syndrome: recognition prevents morbidity and mortality |
Systemic capillary leak syndrome: recognition prevents morbidity and mortality. Abstract: The authors report on a case of SCLS in Australia involving a 61-year-old male who was properly diagnosed after his third episode, to increase awareness of the condition and to highlight the benefits of prophylactic intravenous immunoglobulin (IVIG) in this condition. The diagnosis was made by exclusion and clinically by a classic triad of hypotension, hypoalbuminaemia and haemoconcentration. There have been recent advances in understanding the pathophysiological basis for SCLS and in effective prophylaxis, and the authors and patient benefitted from said advances. |
03/23/2017 | |
SCLS in Children |
Idiopathic Systemic Capillary Leak Syndrome in Children Abstract: Adult subjects with systemic capillary leak syndrome (SCLS) present with acute and recurrent episodes of vascular leak manifesting as severe hypotension, hypoalbuminemia, hemoconcentration, and generalized edema. We studied clinical disease characteristics, serum cytokine profiles, and treatment modalities in a cohort of children with documented SCLS. Six children with SCLS were recruited from the United States, Australia, Canada, and Italy. Serum cytokines from SCLS subjects and a group of 10 healthy children were analyzed. Children with SCLS (aged 5-11 years old) presented with at least 1 acute, severe episode of hypotension, hypoalbuminemia, and hemoconcentration in the absence of underlying causes for these abnormalities. In contrast to what is observed in adult SCLS, identifiable infectious triggers precipitated most episodes in these children, and none of them had a monoclonal gammopathy. We found elevated levels of chemokine (C-C motif) ligand 2 (CCL2), interleukin-8, and tumor necrosis factor α in baseline SCLS sera compared with the control group. All patients are alive and well on prophylactic therapy, with 4 patients receiving intravenous or subcutaneous immunoglobulins at regular intervals. The clinical manifestations of pediatric and adult SCLS are similar, with the notable exceptions of frequent association with infections and the lack of monoclonal gammopathy. Prophylactic medication, including high dose immunoglobulins or theophylline plus verapamil, appears to be safe and efficacious therapy for SCLS in children. |
03/23/2017 | |
High-Dose IVIG Therapy for SCLS |
High-Dose IVIG Therapy for SCLS. Abstract: We evaluated IVIG prophylactic therapy in a cohort of 29 patients with Systemic Capillary Leak Syndrome in a longitudinal follow up study. All patients received treatments at the discretion of their primary providers and retrospectively via questionnaire recorded symptoms beginning with their first documented episode of the SCLS until May 31, 2014. Twenty-two out of 29 patients responded to the questionnaire, and 18 out of the 22 respondents received monthly prophylaxis with IVIG during the study period for a median interval of 32 months. The median annual attack frequency was 2.6/patient prior to IVIG therapy and 0/patient following initiation of IVIG prophylaxis (P = 0.001). 15 out of 18 subjects with a history of one or more acute SCLS episodes experienced no further symptoms while on IVIG therapy. In conclusion, IVIG prophylaxis is associated with a dramatic reduction in the occurrence of SCLS attacks in most patients, with minimal side effects. |
03/23/2017 | |
Mechanistic Classification of SCLS |
Mechanistic Classification of SCLS. Abstract: The authors analyzed circulating mediators of vascular permeability and proinflammatory cytokines in acute episodic sera from 14 patients with SCLS, and sera from 37 healthy control subjects. They monitored barrier function of human microvascular endothelial cells (HMVEC) after treatment with SCLS sera using transendothelial electrical resistance assays. Consistent with their previous study, the permeability factor vascular endothelial growth factor (VEGF) was increased in sera from acutely ill subjects with SCLS. An analysis of samples from one SCLS patient who has not responded to any preventive therapies (and who is a member of this Community), suggests that SCLS may have clinically varying forms, and that within the group of patients with SCLS, different cytokines may mediate the capillary leak. Therefore, quantitative molecular and humanized cell-based assays for humoral mediators of permeability should improve diagnostic specificity for SCLS and enable clinicians to screen for effective therapies. |
03/23/2017 | |
IVIG in SCLS: A Case Report and Review of Literature |
IVIG in SCLS: Report and Review of Literature. Abstract: In recent years, IVIG has become a common first-line prophylactic therapy in most patients with benefits at the dose of 2 gr/kg once a month. Here the authors report the case of a 49-year-old male patient in Italy -- he is a member of this community -- with SCLS treated successfully with a lower dose of IVIG (1 gr/kg monthly) in the maintenance phase. He presented no acute episodes in a follow-up period of 28 months. The authors describe prophylactic treatments for SCLS in the literature and compare their patient to another 18 who received IVIG in follow-up. |
03/23/2017 | |
Genome-Wide SNP Analysis of SCLS |
Genome-Wide SNP Analysis of SCLS. Abstract: Polymorphisms in genes whose functional annotations suggest involvement in cell junctions and signaling, cell adhesion, and cytoskeletal organization, correlate with our previous mechanistic studies of SCLS sera. Such annotations provide a framework for future allelic discrimination strategies to validate top-ranked SNPs discovered here, as well as novel SNPs unique to the SCLS cohort detected by exome capture sequencing. Although the findings must be corroborated in a larger cohort, they provide a springboard for discovery of underlying pathophysiological mechanisms, biomarkers, and avenues for therapy. |
03/23/2017 | |
The Mayo Clinic Experience with SCLS |
Idiopathic Systemic Capillary Leak Syndrome (Clarkson's Disease): The Mayo Clinic Experience Abstract: Of the 34 patients whose records were reviewed, 25 fulfilled all diagnostic criteria for SCLS. The median age at diagnosis of SCLS was 44 years. Median follow-up of surviving patients was 4.9 years, and median time to diagnosis from symptom onset was 1.1 years (interquartile range, 0.5-4.1 years). Flulike illness or myalgia was reported by 14 patients (56%) at onset of an acute attack of SCLS, and rhabdomyolysis developed in 9 patients (36%). Patients with a greater decrease in albumin level had a higher likelihood of developing rhabdomyolysis (P=.03). Monoclonal gammopathy, predominantly of the IgG-kappa type, was found in 19 patients (76%). The progression rate to multiple myeloma was 0.7% per person-year of follow-up. The overall response rate to the different therapies was 76%, and 24% of patients sustained durable (>2 years) complete remission. The estimated 5-year overall survival rate was 76% (95% confidence interval, 59%-97%). In conclusion, SCLS, a rare disease that occurs in those of middle age, is usually diagnosed after a considerable delay from onset of symptoms. The degree of albumin decrement during an attack correlates with development of rhabdomyolysis. A reduction in the frequency and/or the severity of attacks was seen in nearly three-fourths of patients who were offered empiric therapies. The rate of progression to multiple myeloma appears to be comparable to that of monoclonal gammopathy of undetermined significance. |
03/23/2017 | |
Sharing the Pain [of living with SCLS] |
Sharing the Pain [of living with SCLS] This article from The Washington Post newspaper tells the story of how this SCLS virtual community was created, the story of its founder and, more generally, of this fantastic RareShare site.
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03/23/2017 | |
Idiopathic systemic capillary leak syndrome (Clarkson disease) |
Idiopathic systemic capillary leak syndrome (Clarkson disease) Abstract: The enigmatic systemic capillary leak syndrome (SCLS) named for Dr Clarkson is characterized by transient and severe but reversible hemoconcentration and hypoalbuminemia caused by leakage of fluids and macromolecules into tissues. Although less than 500 cases of SCLS have been reported in the literature since 1960, the condition is probably underdiagnosed because of a lack of awareness and a high mortality without treatment. Treatment of acute SCLS remains primarily supportive. Prophylaxis with IVIG appears promising, but this therapy is nonspecific and expensive. Mechanistic understanding of SCLS is in its infancy. As a result, clinicians today cannot predict when or how badly SCLS will flare; targeted therapies do not yet exist, and prolonged remission or cure remains elusive. Our working hypothesis invokes exaggerated microvascular endothelial responses to surges of otherwise routinely encountered inflammatory mediators. This emerging disease model lends itself to innovative patient-centered translational research in the ways highlighted above. It is our hope that detailed and personalized investigation of intraendothelial responses among individual patients with SCLS might illuminate novel genetic and molecular control mechanisms. In turn, such advances could deliver the diagnostic, prognostic, and therapeutic tools sorely needed to combat this devastating disease. |
03/23/2017 | |
Capillary leak syndrome: etiologies, pathophysiology, and management |
Capillary leak syndrome: etiologies, pathophysiology, and management Abstract: In various human diseases, an increase in capillary permeability to proteins leads to the loss of protein-rich fluid from the intravascular to the interstitial space. Although sepsis is the disease most commonly associated with this phenomenon, many other diseases can lead to a “sepsis-like” syndrome with manifestations of diffuse pitting edema, exudative serous cavity effusions, noncardiogenic pulmonary edema, hypotension, and, in some cases, hypovolemic shock with multiple-organ failure. The term capillary leak syndrome has been used to describe this constellation of disease manifestations associated with an increased capillary permeability to proteins. Diseases other than sepsis that can result in capillary leak syndrome include the idiopathic systemic capillary leak syndrome or Clarkson’s disease, engraftment syndrome, differentiation syndrome, the ovarian hyperstimulation syndrome, hemophagocytic lymphohistiocytosis, viral hemorrhagic fevers, autoimmune diseases, snakebite envenomation, and ricin poisoning. Drugs including some interleukins, some monoclonal antibodies, and gemcitabine can also cause capillary leak syndrome. Acute kidney injury is commonly seen in all of these diseases. In addition to hypotension, cytokines are likely to be important in the pathophysiology of acute kidney injury in capillary leak syndrome. Fluid management is a critical part of the treatment of capillary leak syndrome; hypovolemia and hypotension can cause organ injury, whereas capillary leakage of administered fluid can worsen organ edema leading to progressive organ injury. The purpose of this article is to discuss the diseases other than sepsis that produce capillary leak and review their collective pathophysiology and treatment. |
03/24/2017 | |
The Clinical Picture of Severe SCLS Episodes Requiring ICU Admission |
The Clinical Picture of Severe SCLS Episodes Requiring ICU Admission Abstract: SCLS is a very rare cause of recurrent hypovolemic shock. Few data are available on its clinical manifestations, laboratory findings, and outcomes of those patients requiring ICU admission. This study was undertaken to describe the clinical pictures and ICU management of severe SCLS episodes. This multicenter retrospective analysis concerned patients entered in the European Clarkson's disease (EurêClark) Registry and admitted to ICUs between May 1992 and February 2016. Fifty-nine attacks occurring in 37 patients (male-to-female sex ratio, 1.05; mean ± SD age, 51 ± 11.4 yr) were included. Among 34 patients (91.9%) with monoclonal immunoglobulin G gammopathy, 20 (58.8%) had kappa light chains. ICU-admission hemoglobin and proteinemia were respectively median (interquartile range) 20.2 g/dL (17.9-22 g/dL) and 50 g/L (36.5-58.5 g/L). IVIG was infused during 15 episodes (25.4%). A compartment syndrome developed during 12 episodes (20.3%). Eleven (18.6%) in-ICU deaths occurred. Bivariable analyses (the 37 patients' last episodes) retained Sequential Organ-Failure Assessment score greater than 10 (odds ratio, 12.9 [95% CI, 1.2-140]; p = 0.04) and cumulated fluid-therapy volume greater than 10.7 L (odds ratio, 16.8 [1.6-180]; p = 0.02) as independent predictors of hospital mortality. In conclusion, high-volume fluid therapy was independently associated with poorer outcomes. IVIG use was not associated with improved survival; hence, its use in an ICU setting should be considered prudently and needs further evaluation in future studies. |
07/05/2017 | |
Clinical Presentation, Management, and Prognostic Factors of SCLS |
Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review Abstract: A total of 133 case reports (161 patients) and 5 case series (102 patients) of idiopathic SCLS were included in a survey of articles published through end-2016. The findings include that patients had hypotension (81.4%), edema (64.6%), and previous flu-like illness (34.2%). They were often misdiagnosed as having hypovolemic shock, septic shock, polycythemia vera, or angioedema. Thirty-seven patients died (23%) mainly because of complications from SCLS (78.4%). There were significant differences in the survival rates between patients who were treated with prophylactic b2 agonists, methylxanthines, and intravenous immunoglobulins and those who were not. The estimated 1-, 5-, and 10-year survival rate of patients treated with intravenous immunoglobulins was 100%, 94%, and 94%, respectively. The results of this review suggest that prophylactic use of intravenous immunoglobulins is the most effective treatment in reducing the mortality rate of SCLS patients. |
11/04/2018 | |
Idiopathic SCLS (Clarkson syndrome) in childhood |
Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review Abstract: The authors performed a systematic review of the literature on Clarkson syndrome in subjects less than 18 years of age, and identified 24 reports, published since 1989, providing data on 32 otherwise healthy subjects, who experienced 67 well-documented episodes of SCLS. The condition affected more frequently girls (21, 66%) than boys, presented throughout childhood, and was preceded by a mostly viral illness in 75% of cases. The presence of a monoclonal gammopathy (MGUS) was never reported. Uncompensated circulatory shock, muscle compartment syndrome, acute kidney injury, pulmonary edema, and either pleural or pericardial effusion were, in decreasing order of frequency, the most common complications. Four patients died. In sum, SCLS develops not only in adulthood but also in childhood, but of potential significance is that in this age group the condition is not linked to an MGUS, and thus it could be that it does not play a pivotal pathogenic role. |
11/04/2018 | |
Whole Exome Sequencing of SCLS Patients |
Whole Exome Sequencing of Adult and Pediatric Cohorts of the Rare Vascular Disorder Systemic Capillary Leak Syndrome Abstract: The extent to which genetic abnormalities contribute to SCLS is unknown. The authors identified pediatric and adult cohorts with characteristic clinical courses and sought to identify a possible genetic contribution to SCLS through the application of Whole Exome Sequencing (WES). On the basis of 9 adult and 8 pediatric SCLS patients and available unaffected first-degree relatives, they did not identify a uniform germline exomic genetic etiology for SCLS. However, WES did identify several candidate genes for future research. |
11/04/2018 | |
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 | |
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 | |
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 |
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 | |
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 | |
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 | |
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 | |
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 | |
PARP15 is a Susceptibility Locus for SCLS |
PARP15 is a Susceptibility Locus for Clarkson Disease Abstract: PARP15 is an enzyme that regulates cellular proteins; it senses single- and double-stranded DNA breaks and plays a key role in DNA repair and other cellular processes. We analyzed barrier function in PARP15-deficient vascular endothelial cells and vascular leakage in mice, and concluded that several loss-of-function PARP15 variants are associated with SCLS. PARP15 thus represents a previously unrecognized genetic susceptibility factor for SCLS. |
11/18/2024 | |
Systemic Capillary Leak Syndrome: A Primer |
Systemic Capillary Leak Syndrome Abstract: The different forms of SCLS include idiopathic SCLS (ISCLS) and secondary SCLS (SSCLS), which can be triggered by several conditions, including certain infections and haematological malignancies. A subgroup of patients with ISCLS have monoclonal gammopathy-associated SCLS (also known as Clarkson disease), which is an ultra-rare and extreme form of ISCLS. ISCLS can be managed effectively with monthly prophylactic immunoglobulin therapy whereas SSCLS frequently does not recur once the underlying condition resolves or the offending agent is discontinued. Thus, differentiation between ISCLS, SSCLS and other causes of oedema is crucial for quick diagnosis and positive patient outcomes. |
11/18/2024 |
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I founded this virtual community in mid-2008.
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 there is emerging evidence that IVIG should also be administered as soon as possible after the onset of an episode of SCLS.
For my history of this RareShare SCLS community, visit https://rareshare.org/topics/1844, https://rareshare.org/topics/1847, https://rareshare.org/topics/1850, and https://rareshare.org/topics/1851
My personal email address is aporzeca@american.edu
A Dutch PhD researcher working in the area of consumer behaviour, who has been coping with capillary leak since 2019. Had a few acute attacks (all triggered by influenza/vaccination), but it has...
Just diagnosedwith SCLS after my fifth attack in seven years. So happy finally to have an answer and looking forward to preventative treatment,
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...
Wife and mother of four
Fitness Instructor and Personal Trainer
Diagnosed with CLS in Novemeber of 2011. Long hospital stay with facitomies due to incorrect treatment casuing compartment...
My name is Rick and I was diagnosed with SCLS in May 2022. A healthy male for over 59 years who has a history of very little illness throughout my entire life. A lifelong athlete,...
I am the wife of Rick. Rick was diagnosed with SCLS exactly one year ago in 2022. A healthy male for over 59 years who has had little illness to contend with. An athlete, avid...
My name is Cara. I'm 64 years old, married, have 3 adult children, and six grandchildren. I was just diagnosed with SCLS at Mayo Clinic in December 2022 where I sought a consult after my...
I have had 4 hospitalizations since October 2021. My 4th wad end of June 2022 and was out of state in Washington. They diagnosed me with SCLS.
Diagnosed with Systemic Capillary Leak Syndrome after several years of attacks starting around age 46. I'm currentl 52, residing in St. Louis, MO, US.
I also have Meniere's Disease, slight...
Trying to self-diagose and help my doctors figure out what has caused 10 hospitalizations in the last 5 years related to fluid retention. Possible SCLS.
The first episode occurred in April 2019. After an upper respiratory infection, after flying for four hours, the feet were swollen and low blood pressure.
In May 2020, after a long-distance...
50 year old female, no children, healthy life and no previous health problems.
Diagnosed with SCLS on Febrary 2021 after severe episodes since octuber...
Mother to son who has capillary leak syndrome and has been successful with ivig treatmwnt for 10 yrs
Mother to son who has capillary leak syndrome and has been successful with ivig treatmwnt for 10 yrs
Hallo, ich bin Deutsche und 59 Jahre alt. 40 Jahre mit vielen unklaren Symptomen / Krankheiten (Monoklonale Gammopathie, Sklerose, Fibrose...), extreme Schmerzen und vieles mehr. V.a....
Diagnosed with SCLS in February 2021 after what was probably my third attack in 18 years. The trigger the third time was the Moderna COVID vaccine. I am grateful to the doctors who diagnosed me...
My daughter passed away from Clarkson's disease, or so we believe. I hope to get a diagnosis for her post-mortem and to help further understanding of this sydrome.
Hello, my name is Jane and I live in Minnesota, USA. My first episode was in 2003 where I was hospitalized for a week. In 2009, I had my second episode which lead to my diagnosis of...
After 15 years of semi-diagnosed illnesses, my allergist identified I likely have a varient of Systemic Capillary Leakage Syndrome. So I am new to this diagnosis (which at least is more in the...
Diagnosed in March 2011 following 3 Clarkson attacks.
Prise en charge par ivg et suivie par le Pr Levesques du CHU de Rouen.
Born with IgA immunodeficiency, two episodes of Idiopathic Systemic Capillary Leak Syndrome (Clarkson’s Disease).
I was diagnosed CLS in September 2019 while I was on holidays in France. At first stage I was treated in the hospital of Brest (region of Bretagne in France) later on I was...
Ik heb mijn eerste en enigste aanval gehad in januari 2017. Ze hebben toen mijn beide benen en armen moeten openen om de druk van mijn ledematen af te halen. Door de aanval heb ik ook blijvende...
Hi, I am a 53 year, female. I live in Brisbane, Australia. I was originally given a clinical diagnoses of ISCLS in January 2019. It took 18 months to get a confirmed diagnosis....
Diagnosed SCLS on February 21, 2019 at 61 years old
I was diagnosed with SCLS following a second SCLS episode, the first I was in the ICU unit and ventilated/induced coma, at that time the doctors thought it was sepsis. The first episode was in...
Systemic Capillary Leak Syndrome - what's this thing that's taken over my body??Otherwise normally superfit 52 year old. Happy to be involved in any and all research, etc. Frequently travel...
Having read what I wrote here while confined to my hospital room on day three of my current stint for very low hematocrit values (<2.5) (July 2021)
I realize how atrocious what I had...
My husband was diagnosed with SCLS in 2018 after 2 events- one in January 2018 and the first in December 2016. Both events began with flu-like symptoms and he quickly went downhill into acute heart...
I have recently(September 2018) been diagnosed with scls after a 4 week stay in hospital, most of which in intensive care. I had severe swelling around my body but mostly my legs resulting in the...
SCLS - Perth, Western Australia, 28 Male
First attack, Started around 17/11/2017, SJOG Midland Hospital
Flu like symptoms and masssive swelling in legs, guts and arms. Drove to work 12 hrs but...
I have had SCLS for over 8 years and been doing IVIG since June of 2012 checking in first time when I first got this over 30 doctors said I would not survive I am crippled in three of my limbs...
I am a nurse who have a Clarkson syndrome evolving since several years.
At first, the symptomatology was chronic, but gradually, crisis became more and more severe (since October 2017).
I was diagnosed with SCLS in 2018 after many hospitalizations and was started on IVIG in February 2018.
Husband to a patient that is highly suspected to have SCLS as of March 2018 - her doctors have been in contact with Dr. Druey, and he indicated it seemed like a classic presentation of...
I was diagnosed Jan. 2018 .I had an acute attack and ended up in ICU . Fortunately I was diagnosed within three days . I am on no medication and am trying to get an appointment with an immunologist...
I was diagnosed with SCLS in early February of 2018. I suffered my first major leak in December of 2016. The cardiologist at Johns Hopkins at that time saved my life, but did not have a...
I was diagnosed with SCLS in Boston in early 2000 - almost 3 weeks after initially being admitted to the hospital. I felt flu-like and completely lethargic the day I was admitted, to the point...
Just diagnosed in Nov 2017 with SCLS. I am 48 from Canada.
I had my first episode in January 2017. At this time I thought I had the flu virus and didn’t think much of it. After a few days I...
I have been recently diagnosed with smoldering myeloma and edema due to capillary leakage syndrome. The build up of fluid started last spring. I have been put on oral steroids which seems to take...
arielbatt@yahoo.com
Update to October 2018: I have been a little over a year with recommended dose of IVIG, I get tired more than usual, but less than a few months ago, I continued with my...
Married to Ruth with three little scoundrels,Sofia, 10,Luke, 6 and Dan, 5.
Diagnosed in Nov '14 with a devastating attack which required extensive surgery and a 6 week stay in the...
I've been dealing with what appears to be a chronic form of capillary leak condition for several years. It started after a viral infection and became chronic over time. All of the usual causes...
I live in the Uk and would be...
Diagnosed with a chronic presentation of SCLS with recurrent-mild episodes that may escalate to severe episodes that require hospitalization.
Daily maintenance treatment since...
Hello my name is Andrea, I am 45 years old and from Germany. I recieved the diagnoses of Capillary leak Syndrome in march 2016.
Was diagnosed in 2010 at Mayo Clinic by Dr. Phillip Greipp.
First attack 1998 (18 more attacks before diagnosed. First in 98, next not until 2004, increased in frequency until 2010 for total...
I am married to Cristiano, my husband was diagnosed with SCLS february 2016. We live in Denmark with our 2 children (10 and 13 years old).
• First episode:
October...
Born 1974, live in Germany near Stuttgart, married, have two young Kids, damaged legs (fcopmartment syndrome, asciotomy) and feet, fine motor skills of my hands are bad (criticall Illness...
Keen sports fan and...
But I due to my condition I go back and forth.
3 years ago I had a rough time in my life a year full of stress,
...
My first attack was 3/2000. RAK amputation, nerve damage in both hands & left foot. In hospital for 4 months.
Hello,
My name is Annmarie and I am a 57 year old female and live in Sydney, Australia.
I am suffering from a proposed mild but very chronic version of Capillary Leak Syndrome. I suffer...
Female Age 48 - very fit and healthy previously . Experienced first episode Sept 2014 and been having repeat attacks on average 1 per month ( but nor clearly linked to hormonal cycles) since then....
first attack at 8 years of age. It took us three years to get a diagnosis. Has been on IVIG since February 2015 and is doing very well, no attacks since Juli 2014
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...
2009 is when she had her first episode after a...
I would like to join your community to find out more about it and add my...
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 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...
My wife, Jen, is also a member of the site, but I...
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...
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....
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...
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...
Before that my first "crisis" started in summer 2010...
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...
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...
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
...
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...
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...
Ela has had 4 episodes in the past year (September 2010 to date). 3...
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 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...
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...
Shannon has just been admited into Cardiac Care to monitor her current episode. We have been fortunate to have the...
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...
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 was initially put on Theophylline and Prednisone with one minor recurrence...
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 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...
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...
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 husband's first episode:
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...
I suppose I'm on a life...
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 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.
...
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...
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...
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...
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...
First acute episode of systemic capillary leak syndrome on May 2003. Failure of different treatments and 5 new...
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.
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...
She has had a difficult time recently, with admissions to ICU and the upset...
I founded this virtual community in mid-2008.
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...
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