High dose intravenous immunoglobulin therapy of the Systemic Capillary Leak Syndrome (Clarkson disease).

[systemic capillary leak syndrome]

The Systemic Capillary Leak Syndrome is a highly rare disorder of unknown etiology . 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 IVIG prophylactic therapy in a cohort of 29 patients with Systemic Capillary Leak Syndrome in a longitudinal follow up study .All patients received treatments at the discretion of their primary providers and retrospectively via questionnaire recorded symptoms beginning with their first documented episode of the Systemic Capillary Leak Syndrome until May 31 , 2014 .Twenty -two out of 29 patients responded to the questionnaire , and 18 out of the 22 respondents received monthly prophylaxis with IVIG during the study period for a median interval of 32 months . The median annual attack frequency was 2 .6 /patient prior to IVIG therapy and 0 /patient following initiation of IVIG prophylaxis (P = 0 .001 ). 15 out of 18 subjects with a history of one or more acute Systemic Capillary Leak Syndrome episodes experienced no further symptoms while on IVIG therapy .IVIG 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 IVIG for the Systemic Capillary Leak Syndrome and to determine optimal dosage and duration of therapy .