I have not had an attack for over 2 years, then BANG!  A severe attack with hypotension, shock, haemoconcentration, hypoalbuminemia, AND most importantly, SEVERE myalgia.  It came on at 5am and was gone by 7.15am, by which time all around were panicking.  My IgG levels fell from 24g/l to < 18g/l.

Now, I cannot understand why, all of a sudden.  Was it due to a recent chest infection?  Was it due to the steroids used to treat the chest infection(50mg/day for a week, and tapering)?  Or was it due to a bad batch of IVIG? Or was it something else?  Why would my IgG levels fall so unusually?

My guess is that any chest infection in my case, should be quickly treated with antibiotics, even at the risk of overuse.  Has anybody had experience with  cortisone induced scls? Or cortisone reducing the blood levels of IVIG?   Or a suspect batch of ivig.

john

John,

So sorry to hear about this scary incident!  Would you kindly provide additional details? Had you received IVIG the day before this happened?  How did you know something was wrong at 5 AM?  (It was the muscle pain (myalgia), I presume.)  Did you go to a hospital ER between 5 and 7:15 AM?  (I presume that's where you got all those blood readings.)  (If so, congratulations on the speed with which they moved, by the way!)  And what if anything did you have them do?  To give you still more IVIG?  And when you say 7:15, you mean that is when you got new readings which were normal, or how else did you determine that the situation had been improved or resolved?

As to your questions, many of us have taken steroids like oral Prednisone or been injected Solumedrol (methylprednisolone) before, during or after episodes of SCLS, or have taken oral Prednisone before, during or after infusions of IVIG, with the purpose of helping matters (e.g., aborting an episode of SCLS or preventing allergic reactions to IVIG), but I've never heard about steroids inducing SCLS or making matters worse; on the contrary!

 

Dear John,

I'm sorry to hear your recent story.

Despite current research, no serious progress has been made on the cause of the onset of capillary leak. To my knowledge, oral or injected Cortisone never triggered a leak. On the contrary, a viral infection of the upper or lower respiratory tract sometimes seems to precede a leak. This is not to say that an infection is its cause ! In my case, I had for 14 years many pharygites, sinusitis, bronchitis, without triggering a leak. Similarly, I occasionally take oral cortisone for osteoarticular problems, without ever triggering a leak. Finally, I was operated multiple times (reconstruction of a shoulder, knee prosthesis, posterior lumbar intersomatic fixation ...) without ever a leak.  I had to change immunoglobulins several times for logistical reasons (Redimune, Octagam, Kiovig, Privigen…) without any new leak. In my case, all different treatments used are effective in terms of protection against an acute leak. Since the beginning of the treatment with immunoglobulins (2005) I had only a small leak in October 2008, directly after an influenza vaccine (???). You write "My IgG levels fell from 24g/L to < 18g/L". I believe that normal IgG rates are between 6.7 - 12.5 g/L. I do not know how to interpret the numbers you give. In my case IgG values are between 12.1 and 16.3 g/L just before IvIg. 

Anyway my treatment is well supported, effective, with few side effects and currently normal renal function, no amyloidosis, no myeloma nor lymphoma.

 

I address you both, dear John et dear Prof. Arturo, my best regards.

Claude and Arturo, thankyou.

I will gladly expand, as it is perplexing.   

I have been having fortnightly IVIG for the past 4 years.  There have been times that I have extended the interval to 19 days, without incident.  At all times, I have checked my 'trough' level of serum IgG, and the lowest it has been is 20g/l.  So, I was led to believe that as long as I received my standard IVIG dose within 19 days, I would be safe.

However, this October, I took a small break, and on day 16 after my standard dose, I came down with the attack described. (More later).   The only difference was that, in those 16 days, I also received antibiotics and steroids for a chest infection. I was taken to a small rural health centre where my IgG level was 18g/l, whereas 16 days prior it was 24g/l.  So issue #1 is, why did my IgG level drop suddenly?, and what (if known) is a safe level?   I have always believed in the assumption that anything over 12-14g/l was safe, and certainly over 16g/l would be ok.  Thus I search for an answer.  Now we know that steroids drop the serum level of IgG,over time,  but is this significant? i am not convinced.  Issue #2 is, "What is the role in the use of steroids during an acute episode? ".  I have always taken 50mg prednisolone while waiting for the ambulance, but feel silly doing so as it doesn't act in a short time period, and it's action may well be adverse.  I will continue to take the steroids while waiting for the ambulance though.

The third issue relates to the episode itself.  What has become apparent over time is that the presenting symptoms vary so much.   Some will present with oedema, while others shock etc.  My first episode started with myalgia, but rapidly progressed to shortness of breath as the pulmonary oedema took over.  With subsequent episodes, I recognised the myalgia, and called for help early.  The pattern has a sudden onset and cessation. This episode woke me at 5am with muscle pain.  By 5.05, I couldn't stand unaided, and we had called the ambulance.  Every muscle in my body was in spasms.  My masseter jaw muscles cramped, and I could only talk through my teeth.  I was unable to hold my cell phone.  My wife helped me to the car where I got the most relief from the heated car seats.   As this was in a rural setting, the ambulance had some 120km to travel, and the delay was difficult.  My oximeter was reading 85% and falling.  I didn't have my sphygmomanometer with me.   However when the paramedics arrived at 6.30, the attack was nearly over.   I have an A6 laminated crib sheet which gives a treatment plan, names and numbers of relevant Physicians, medicare numbers, allergies etc.  (I carry this everywhere on my smart phone).  The obverse also details what investigations and blood tests need to be ordered.  Essentially, I get an IV line with minimal crystalloids.  (This time was 1l over 5 hrs), high flow O2 or CPAP if available, pain relief, and antibiotics.  By 7am the attack was over from my point of view.  My breathing was normal, the pain had gone, and I was able to sit up in ED and tell them my BP, pain, and Sats had turned the corner.  My wife has got used to this, but everybody else is very sceptical for the next 24 hrs!   Just a side issue here is that I was a 2-3 hour flight to the next tertiary hospital, and the threat of acute pulmonary oedema demanded a pressurised cockpit!  Road transport over bumpy rural roads is not on my agenda.  Somehow, I feel this makes the condition worse.

Claude, you seem to be bionic!   Do you fluoresce at night?  I do confess that my dosage rate appears higher than many, but I loathe to try a change.  It does however point to a multifactorial origin, -that there is not single cause.  I have discussed with my physicians the possibility that in my case, the IVIG works through a hyperosmolar effect.   I am heartened by your tolerance of the steroids.

Arturo, in all my 4 episodes, I have never had IVIG during an acute attack, as it seems to be self-limiting every time.  My first attack had severe shock, pulmonary oedema, sats 65%, Hb 220, low albumin, and lasted from 6.15am to 10.45am, before I new I was coming right.  At 11am, I still needed CPAP, and volume expansion for hypotension, but I could tell the episode was over..  I stay in ICU afterwards to prevent the resorption phase, and ensure renal function is OK

So, thankyou kind folk.  I am reassured by the steroid situation, puzzled by the Hydra that is SCLS, and have only this to offer.... That a treatment plan worked well for me.. Either laminated or on the phone.

Kind Regards

John

FYI new research on info will be published soon. No new treatment options but interesting information.