[A case report of systemic capillary leak syndrome. Effective treatment with immunoadsorption and leukotriene...

Systemic capillary leak syndrome (SCLS) is an unusual condition characterized by periodic leakage of plasma proteins through the capillary wall, leading to hypoalbuminaemia, hypovolaemia, haemoconcentration and shock. The pathogenesis is unknown and the mortality high. Various prophylactic treatments have been tried but are difficult to evaluate because of the unpredictable course of the disease. We describe one patient with frequent attacks of SCLS, that did not respond to any kind of treatment regimens over a period of nine years. Subsequently, therapy with regular immunoadsorption of plasma proteins and a leukotriene receptor antagonist was attempted. This treatment has now been given for six years and only one episode of capillary leakage has occurred. The effect may be due to removal of pathogenic immunoglobulins or immune complexes and/or inhibition of leukotriene-induced capillary leakage.