Systemic capillary leak syndrome: recognition prevents morbidity and mortality.

Idiopathic systemic capillary leak syndrome (SCLS) is extremely rare but carries a high morbidity and mortality. The diagnosis is made clinically by a classic triad of hypotension, hypoalbuminaemia and haemoconcentration. There have been recent advances in understanding the pathophysiological basis for SCLS and in effective prophylaxis. We report a case of SCLS to increase awareness of the condition and to highlight the benefits of prophylactic intravenous immunoglobulin in this condition.

Rabbolini DJ ，Ange N ，Walters GD ，Pidcock M ，Randall KL ... -  《-》

High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome.

Lambert M ，Launay D ，Hachulla E ，Morell-Dubois S ，Soland V ，Queyrel V ，Fourrier F ，Hatron PY ... -  《-》

High-dose intravenous immunoglobulins: a promising therapeutic approach for idiopathic systemic capillary leak syndrome.

Zipponi M ，Eugster R ，Birrenbach T 《-》

Clarkson disease - systemic capillary leak syndrome in a 6-year-old girl: case report.

Kerketta J ，Lodh M ，Mandal K 《-》

Severe capillary leak syndrome with cardiac arrest triggered by influenza virus infection.

Systemic capillary leak syndrome (SCLS), also known as Clarkson syndrome, is a rare disease with potential fatal outcome. The clinical picture involves leakage of fluid and protein from the bloodstream into peripheral tissues, resulting in hypoalbuminaemia, elevated haematocrit, oedema and hypotension. The spectrum of the symptoms ranges from discrete swelling/oedema of extremities to fulminant cardiogenic shock. We present a case with a 52-year-old man diagnosed with SCLS after being resuscitated from cardiac arrest, which was complicated by compartment syndrome. The severe episode of capillary leak was potentially triggered by influenza virus infection. With the benefit of hindsight, he presented with symptoms of SCLS 2 years prior the major acute episode. Here we describe this case and review some aspects of the literature on SCLS, with particular focus on the pathogenesis, treatment/prophylaxis and long-term physical and psychological complications.

Ebdrup L ，Druey KM ，Druey K ，Mogensen TH ... -  《-》