Idiopathic systemic capillary leak syndrome presenting as septic shock: A case report

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Background:Idiopathic capillary leak syndrome (Clarkson's Disease) is a rare angiopathy with a heterogenous phenotype that may present as distributive shock refractory to resuscitative management.Objective:We report a case of idiopathic systemic capillary leak syndrome presenting as septic shock.Methods:Structured case report and review of the literature.Results:A 27-year old man admitted to our institution with coryzal symptoms rapidly deteriorated with presumed sepsis, leading to intensive care unit admission. Following further deterioration, Idiopathic systemic capillary leak syndrome was considered and intravenous immunoglobulin administered, resulting in rapid improvement in the patient's clinical status.Conclusions:Idiopathic systemic capillary leak syndrome is a rare and potentially life-threatening angiopathy that may present as, and should be considered in, refractory distributive shock. Administration of intravenous immunglobulin resulted in rapid recovery in this patient, and has been associated with positive outcomes in previous cases.

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