IDIOPATHIC CAPILLARY LEAK SYNDROME COMPLICATED BY MASSIVE RHABDOMYOLYSIS

Idiopathic systemic capillary leak syndrome (Clarkson's disease) is ch aracterized by recurring attacks of increased capillary permeability, resulting in severe hypovolemic shock due to plasma extravasation. Add itional laboratory features include association with a monoclonal gamm opathy, extreme hemoconcentration, and hypoalbuminemia. Rare manifesta tions of this syndrome are renal damage and rhabdomyolysis due to incr eased compartment pressure and ischemic myonecrosis. We present the fi ndings in two patients with capillary leak syndrome complicated by sev ere rhabdomyolysis, in one case leading to acute renal failure. We rev iew therapeutic aspects of this rare syndrome and emphasize the import ance of early diagnosis and of prompt and aggressive fluid replacement .