Idiopathic systemic capillary leak syndrome: Novel therapy for acute attacks

Patients typically present in the fifth and sixth decades of life with intermittent acute attacks of peripheral edema, hypotension, hypoalbuminemia, hemoconcentration, weight gain, and a monoclonal gammopathy.1,2 Rhabdomyolysis, compartment syndrome, and acute renal failure are common clinical findings; however, pulmonary edema is rare.3 During an acute attack, there is an unexplained capillary hyperpermeability with a shift of plasma from the intravascular to the interstitial space.4 Acute attacks are followed by a resolution phase, with fluid shifts leading to intravascular volume overload and associated severe pulmonary edema. The usefulness of theophylline and terbutaline in this syndrome, both acutely and for prophylaxis, likely relates to their ability to increase cAMP and thus decrease endothelial cell permeability. [...]we propose a 2-step approach for treatment of acute attacks of ISCLS.