I'm a new member with a new SCLS diagnosis and a history of (now) 6 attacks in the last 7 years. I've been monitored for MGUS for the last five years. The first five attacks, which defied all efforts at diagnosis, were pretty much identical: prodrome of malaise, fatigue, sudden retching; then c. 3 days of paralyzing weakness (can't move, walk); then fluid overload with 12-16 lb. weight gain over a week; then urinating it out over c. a week with gradual recovery of strength and stamina. The triggers for the first five pre-diagnosis attacks were infections: gastroenteritis, influenza A, upper respiratory disease. I was finally diagnosed a month ago and a week ago today began my first ivig infusions, 35 mg a day on 4 consecutive days to test my tolerance. After the third infusion (i.e., after 105 mg) on Thursday the familiar prodrome took hold--feeling sick, crashed at 8 pm, retching at midnight. I was wiped and weak in the morning but at the hospital for the final fourth infusion which was aborted given my presentation: BP 80/50, elevated HCT and Hgb, low albumin, weak but much milder than the five times before. Fluid overload with weight gain began over the weekend and continues. 

My hematologist confirms it was a sixth SCLS attack with two differences from the first five: unknown trigger and much milder in acute phase, maybe because the 105 mg of ivig moderated it. My question for Arturo and the community is, has anyone heard of SCLS triggered by either, a) ivig infusions themselves, or b) ivig flu-like side effects the SCLS attacker mistook for an infection.

Baine 

Dear Baine, Welcome to our Community!

I'm so sorry that it took this long for you to get the right diagnosis, but you're very, very lucky that you survived all those episodes, and apparently without lasting damage to your extremities and organs!

The answer to your questions is No, as far as I'm aware, but for your safety I suggest that you have your hematologist contact either Dr. Druey or Dr. Pecker for a complimentary consultation.  Their contact informatrion is in the Community Details page, under the tab Tips or Suggestions.

I hope that you will be put on a regular, every-4-weeks, preventive therapy of IVIG at the recommended 1 gr/kg per day for two consecutive days.  It's still by far the best way we know to prevent, minimize and treat episodes of SCLS.

Arturo, thank you. I'm honored by membership in the community you founded.

Arturo,  I have been on IVIG for 14 months and have been on 2 grams/kg during that time.  You mentioned above that 1 gram/kg is recommended.  I have seen bunch of people post in the past on other chains that they are also on 2 grams/kg.  I am wondering if anyone has gone from the 2 grams to 1 and have had any subsequent episodes?  Dr Druey did tell me that at first it wouldn't hurt for me to be on 2 gram/kg and then eventually move to 1 but I am a little weary of that move as my two episodes were very life threatening and I had multiple fasciotomies each time and don't want to go through that again.  I haven't had any issues with the 2 grams/kg.

Hi all. Based on what I've seen here and my own experiences, I think there is some variablity to what's recommended.

I was told initially that I'd go on the highest dose possible (2g/kg) and that "eventually" it'd be reduced. After about two years, and after I asked for it to be reduced because missing two days of work a month was proving challenging, Dr. Druey readily agreed. He even said, "Why didn't you ask me sooner?!" We went to 1g/kg.

Somewhat recently, I'd been feeling quite off during and after treatments, and I began to suspect that the treatments were triggering minor leaks, which I thought was crazy. But then Dr. Druey confirmed that that could be happening, and he said I could consider lowering the dose to .8g/kg. But then he said I could also consider raising the dose to 1.25g/kg. He basically said that there's some trial and error here because we don't really know how IVIG works.

I do remain very concerned that the prophylactic treatment meant to reduce the frequency and severity of SCLS episodes might actually be triggering (albeit very minor) SCLS episodes. Logically, that just doesn't seem right or good.

I've been doing .8g/kg for several months now and I've been fine. But if I do have any more minor leaks after treatments I'm going to seriously reconsider IVIG. This will be very, very, very difficult to do without being able to consult Dr. Druey since he's retired.

Has anyone had any luck with Dr. Lyons in San Diego? It's my understanding he won't talk to us unless we go out to San Diego, which is prohibitive for many. Dr. Druey always responded to my emails.

Stay well everyone! Jen

I truly think this is probably one of those "Your mileage may vary" situtations. Due to some possible miscommunications about my IVIG dosage, I ended up on about 0.7-0.8 g/kg which I get in one day (about 5.5 hrs). Dr. Druey was kind of surprised I ended up on that low of a dose. But on the otherhand, I think I have responded well. Within 3 days of my first IVIG treatment, I was feeling orders of magnitude better, was able to hike in the mountains, and just felt better than I had in years. I have not had any attacks since treatment started (well even a 11 months before treatment luckily) Dr Druey was ok with staying on that dose if everything was going well.   But also, my attacks before treatment were probably less severe than average.  They still kept me hospitalized for several days, but never any major complications or fasciotomies. 

The only odd hitch is that my Meniere's like attacks have gotten more frequent ( changes in my hearing for a few days, followed by severe a vertigo attack that lasts about 8 hours).  They used to happen about once every few years, now they happen like clockwork at 3 weeks after an IVIG treatment. It could be the IVIG wearing off and triggering a small leak which happens in my inner ear, or some sort of auto-immune reaction.

A specialist otolaryngologist has prescribed an off-label medicine called "betahistine" that I've been on for a few months now and it has greatly lessened the ear issues.  I'm going through it right now and my next IVIG is Thursday. But the issues are a lot less bothersome than before.   In the future I think I still have the option of upping the IVIG dosage, and/or getting it every two weeks to see if it helps.

I see that Rick misunderstood what I wrote: "the recommended 1 gr/kg per day for two consecutive days," is the same thing, though more precise, as writing "the recommended 2 gr/kg every four weeks over two consecutive days."

Now it is a fact that many SCLS patients are on lower doses, as the cases of Jen and Kevin illustrate. 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 in countries with government-run healthcare systems, where physicians are under pressure or instructions to limit the prescription of expensive medications like IVIG.

There is one medical study published in 2022 that reported the result of experiences in Europe with the reduction ("tapering") and withdrawal of IVIG in SCLS patients, see the third article listed on our Disorder Resources page, https://rareshare.org/communities/systemic-capillary-leak-syndrome#disorder_resources

The summary says: "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."

In other words, patients who were tapered tended to experience more frequent relapses, and those who stopped getting their IVIG tended to die sooner.

Their recommendation: "In summary, IVIG withdrawal is associated with increased mortality and a higher rate of recurrence in SCLS patients. Given this information, a lifelong treatment with IVIG seems ines­capable in Clarkson disease, but dosage tapering might be cautiously considered in stable patients."

If anyone would like a PDF of the article to read and/or share with their doctor, send me an email message to aporzeca@american.edu

I am responding to Baine's question about IVIG causing a capillary leak. I have been on IVIG for 13 years. My first introduction to IVIG was a low dose and not frequent enough to prevent CLS episodes. It took about a year to find the correct amount and frequency which was 2g/kg spilt into two treatments a month. This was effective for over 9 years. After contracting COVID (4x between 2020 - 2023) and getting the vaccine and boosters, I sarted having CLS leaks after each IVIG treatment in 2021. Some were small leaks that corrected themselves without hospital care, and many sent me to the hospital. After consulting with Dr. Druey, I switched to subQ IVIG treatments in 2023, still on 2g/kg. The leaking after treatments was less frequent. Unfortunately, I had a major attack in Oct. of of 2023, and one in Jan. of 2024. However, I am doing much better now. I am not having any leaking after infusions and my energy level is back to normal. I have had many episodes that appeared to not be preceeded by an illness or infection, they seemed to come out of nowhere. Perhaps I had contracted COVID or was exposed to some other illness that showed no symptoms yet sent me into an episode. Perhaps a prior illness or the vaccine and boosters had weakend my capillaries and the amount of fluid that comes with an infusion was too much for my capillaries to handle. No matter the cause, IVIG is what kept me alive.  I continue to do subQ IVIG treatments, and when an episode sends me to the hospital, they treat me with IVIG intraveinously along with albumin and vaso pressors. All this to say, there are many variables that send each of us unique CLS patients into an episode. None of us are the same or respond the same to treatments. As Dr. Druey once said to me, "everything you and your doctor decide to do for your care is an experiment. Report back to me the results of what you try that works and what does'nt".  The only medication he was adamant on as part of my treatment was IVIG for it's effectiveness.  I wish you the best in finding what works for you. I hope we all continue to share our experiences and continue to learn from each other. 

Cristina

My very first IVIG infusion was done in one day over eight hours. It triggered a severe headache which resulted in an ER evaluation with CT scan.  A day later I had symptoms of a leak which did resolve over 24hrs. 

My diagnosing hematologist from Mayo clinic was consulted and he took it to his team. They concluded the leak was triggered by the IVIG infusion. They advised splitting my infusion over two consecutive days. It's now 18 months later without any more post infusion leaks. Over this period, I've had maybe 4 very minor leaks that resolve on their own within 24 hrs.  I had a hig test this past week after contracting COVID for the second time. 19 months ago, it was COVID that provoked a severe leak requiring 8 days of hospital care and ultimately lead to my diagnosis of SCLS after 5 years of mystery attacks. I'm happy to report I had a mild case of COVID and not even a hint of a leak. 

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

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

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!