Idiopathic systemic capillary leak syndrome (clarkson disease)

Idiopathic systemic capillary leak syndrome (SCLS) or “Clarkson disease” is a life-threatening disorder characterized by microvascular hyperpermeability-related recurrent “attacks” or “flares” of severe hypotension, edema, hemoconcentration, and hypoalbuminemia. A monoclonal protein, detected in the majority of the patients, serves as a valuable clue for the diagnosis of SCLS, a disorder with myriad non-specific prodromal and acute attack-related symptoms. Prompt recognition and timely institution of appropriate supportive care are critical. Restoration of normal perfusion requires judicious use of vasopressors, fluids, and occasionally diuretics. Prophylactic therapy with beta-2 agonists, methylxanthines, or intravenous immunoglobulin may reduce the frequency and severity of “attacks.” Although in the recent years we have gained a better understanding of the biology of this perplexing disorder, there remains a paucity of high-level evidence, particularly with regard to efficacy of the currently available therapies. This chapter outlines the diagnostic dilemmas and current approaches related to the management of idiopathic SCLS.