Pulmonary infections associated with systemic capillary leak syndrome attacks in a patient with hypogammaglobulinemia.

[systemic capillary leak syndrome]

Systemic capillary leak syndrome (SCLS ) is a rare disorder of unknown etiology , characterized by recurrent hypovolemic shock attacks associated in most cases with a serum monoclonal immunoglobulin . Prophylactic therapy is usually disappointing and the outcome is often fatal . We report on a patient with recurrent hypovolemic shocks consistent with the diagnosis of SCLS associated with severe serum panhypogammaglobulinemia but no detectable monoclonal immunoglobulin or B cell proliferation . Attacks were often preceded by severe respiratory infections . Both infections and attacks were successfully prevented by i .v . gammaglobulin replacement . Further evaluation is needed to assess the efficacy of i .v . gammaglobulins in patients with SCLS but without hypogammaglobulinemia .