I have listed under Disorder Resources a recently published and very comprehensive article on SCLS titled *"The Systemic Capillary Leak Syndrome: A Case Series of 28 Patients From a European Registry"* which has appeared in the April 5 2011 issue of the prestigious _Annals of Internal Medicine._
It reports on the evolution of 28 patients never previously described in a medical journal, treated mainly in France as far back as 1997 and through mid-2010, one of whom is a member of this Community. Their characteristics were: a median age at disease onset of 49 years (with a range from 5 to 78 years old). The median age at disease onset was higher in men than in women (59 vs. 46 years).
Twenty-six patients were white and 2 patients were black; one was a child; and none had a family history of SCLS. Twenty-five of the patients (89%) had the typical MGUS protein (Monoclonal Gammopathy of Undetermined Significance).
Eight of the patients (29% of total) died at some point, including the child, with death directly related to a severe episode of SCLS in six instances. None of the 8 patients who died were receiving treatment for SCLS, including the child.
Common triggers for attacks included infections (mostly upper respiratory tract infections) in 20 patients (74%) and sustained physical effort in 2 patients (7%). Four of 15 women (27%) experienced attacks during their menstruation.
Of the 23 patients who received medications, 12 were on theophylline and/or terbutaline and/or a similar drug, and 18 patients received IVIG infusions -- because some patients received both types of medications at some point or another.
The study's results suggest that, while not effective right away or in all cases, IVIG is the better course of treatment for SCLS. To begin with, none of the patients receiving IVIG ended up dying, so this medication definitely meets the "first, do no harm" Hippocratic criterion. (The other way to look at it is that IVIG may well have prevented death in all SCLS patients receiving it.)
Of the 14 patients who received the widely recommended dose of 2gr/kg/month of IVIG, only one experienced a single severe episode of SCLS afterwards; the rest either had no episodes (6 patients) or only light episodes (7 patients).
Virtually all those who had one or more mild episodes while on the recommended dose of IVIG had fewer, far fewer, or at least milder episodes than before they went on an IVIG therapy. Even 2 of the 3 patients receiving an inferior dose of IVIG had no more episodes of SCLS.
I recommend that you bring this summary and the article to the attention of your main medical doctor. (Doctors can usually download the article for free, but anybody who cannot do so should write to me at _aporzeca@american.edu_ and I will send it to you.) This article should make it easier for patients who are still having episodes of SCLS to obtain approval and/or medical insurance coverage for monthly IVIG infusions.