Systemic capillary leak syndrome. Case report.

[systemic capillary leak syndrome]

We here report the case of a patient with systemic capillary leak syndrome (SCLS ). This syndrome is a rare condition characterized by recurrent episodes of hypotension with hemoconcentration and hypoproteinemia . It is due to unexplained episodic capillary hyperpermeabilty that results in fluid and protein shift from the intravascular to the interstitial space : generalized edema , shock and renal failure follow . A 59 yo man was admitted to our intensive care unit because of unexplained shock with hemoconcentration , renal failure , and metabolic acidosis . Previous attemps to reverse shock in a medical ward with crystalloids and dopamine failed . An abdominal CT scan , a TEE , and chest X ray study were inconclusive . No sign or history of major infections or anaphylaxis were present . The patient was resuscitated with massive fluid infusions and norepinephrine on the guide of a Swan Ganz catheter . The diagnosis was made on the basis of a previous episode of severe shock complicated with renal failure and a compartment syndrome , the hemoconcentration , and the negative cardiopulmonary findings . A small amount of monoclonal immunoglobulin G , kappa chain , found in the serum confirmed the diagnosis . The SCLS should be considered in the differential diagnosis of idiopathic and anaphylactic shock . Patients may benefit from a prophylactic treatment with theophilline and terbutaline .