Systemic capillary leak syndrome: recognition prevents morbidity and mortality

Abstract

Idiopathic systemic capillary leak syndrome (SCLS) is extremely rare but carries a high morbidity and mortality. The diagnosis is made clinically by a classic triad of hypotension, hypoalbuminaemia and haemoconcentration. There have been recent advances in understanding the pathophysiological basis for SCLS and in effective prophylaxis. We report a case of SCLS to increase awareness of the condition and to highlight the benefits of prophylactic intravenous immunoglobulin in this condition.

Keywords: intravenous immunoglobulin; oedema; polycythaemia; shock; systemic capillary leak syndrome.