I am pleased to report that a group of European physicians is publishing, in the Journal of Allergy and Clinical Immunology: In Practice, the results of a first systematic survey of the experience with reducing the dosage of IVIG, and of ending the administration of IVIG, in patients with SCLS. It will be titled "Intravenous Immunoglobulins Tapering and Withdrawal in Systemic Capillary-Leak Syndrome (Clarkson Disease)."

What is already known is that extended treatment with intravenous immunoglobulins (IVIG) has been associated with fewer recurrences and improved survival of patients duly diagnosed with Systemic Capillary Leak Syndrome.  Compared to IVIG, all other treatments reported in SCLS have a lower level of evidence, a lower efficacy, and a higher rate of side effects.  However, the optimal treatment dosage and duration of such preventive treatment has been unknown.

This forthcoming article adds to our knowledge because it documents that IVIG withdrawal is associated with increased mortality and higher rates of recurrence in SCLS patients.  Therefore, it recommends that IVIG treatments should not stopped in patients with SCLS, although they "could be cautiously tapered in very selected stable patients."

Specifically, they 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IVIG was withdrawn at least once in 18 (31%) patients (W+ group) and never in 41 (69%, W- group).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.

Therefore, the one statistically significant finding is that IVIG withdrawal was associated with increased mortality and a higher rate of recurrence in SCLS patients.  While the association between IVIG withdrawal and worse outcomes was eloquent, there was no such association when considering tapering of dosages.

A link to the soon-to-be-published article will appear under Disorder Resources.  If anybody would like to receive a copy of the preliminary version of this forthcoming article, which will undergo additional copyediting, typesetting and review before it is published (likely in September), write to me at aporzeca@american.edu  

Thanks for the heads up, Arturo.

While I find this study encouraging, as an attorney, hospital administrator, and patient I find this concerning. It is never safe to rely upon one article in order to alter treatment or protocol. While this study is encouraging, let's not take undue risks as patients nor allow insurance companies jump on an excuse to deny coverage. We fought too hard to have IVIG covered.

Susan Tedrick, JD

This important article has now been published in the November 2022 issue of the Journal of Allergy and Clinical Immunology: In Practice, and anyone who would like to receive a copy can request it from me at aporzeca@american.edu