Abstract
Background
Systemic capillary leak syndrome is a highly rare disorder of unknown cause. The disease
is characterized by episodes of transient vascular collapse, which leads to hypotensive
shock and anasarca. Previous treatment of this potentially devastating condition has
been largely ineffective. We evaluated intravenous immunoglobulin prophylactic therapy
in a cohort of 29 patients with systemic capillary leak syndrome in a longitudinal
follow-up study.
Methods
All patients received treatments at the discretion of their primary providers and
retrospectively via questionnaire-recorded symptoms beginning with their first documented
episode of systemic capillary leak syndrome to May 31, 2014.
Results
A total of 22 of 29 patients responded to the questionnaire, and 18 of the 22 respondents
received monthly prophylaxis with intravenous immunoglobulin during the study period
for a median interval of 32 months. The median annual attack frequency was 2.6 per
patient before intravenous immunoglobulin therapy and 0 per patient after initiation
of intravenous immunoglobulin prophylaxis (P = .0001). A total of 15 of 18 subjects with a history of 1 or more acute systemic
capillary leak syndrome episodes experienced no further symptoms while taking intravenous
immunoglobulin therapy.
Conclusions
Intravenous immunoglobulin prophylaxis is associated with a dramatic reduction in
the occurrence of systemic capillary leak syndrome attacks in most patients, with
minimal side effects. A prospective, randomized trial may be necessary to fully assess
the benefits of intravenous immunoglobulin for systemic capillary leak syndrome and
to determine the optimal dosage and duration of therapy.
Keywords
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Article info
Publication history
Published online: September 02, 2014
Footnotes
Funding: This work was supported by the Intramural Research Program of the National Institute of Allergy and Infectious Diseases, National Institutes of Health (Project AI001830, KMD).
Conflict of Interest: None.
Authorship: All authors had access to the data and played a role in writing this manuscript.
Identification
Copyright
Published by Elsevier Inc.
