Systemic capillary leak syndrome - unusual cause of myalgia and elevated creatinkinase

Systemic capillary leak syndrome is a rare idiopathic disorder characterize d by recurrent episodes of hypotension and hemoconcentration due to sudden transient extravasation of up to 70% of plasma. Less than 40 cases have app eared in the literature. Mortality rates 5 years after diagnosis have been reported to be up to 75%. Clinical findings are generalized edema and intra vasal fluid deficiency. Laboratory features include hypoalbuminemia and ext reme hemoconcentration. A common finding is a monoclonal gammopathy. Rare m anifestation of this syndrome is rhabdomyolysis due to increased compartmen t pressure and ischemic myonecrosis. Early diagnosis and exclusion of other clinical disorders like rheumatic myopathies and myositis are critical con cerning prognosis and therapy. We describe a 46-year-old woman with rhabdom yolysis and systemic capillary leak syndrome whose condition is stable for a total of 30 months under a prophylactic regimen with oral terbutaline plu s theophylline. A brief survey of the literature is given.