• Eamon P Raith, Joshua F Ihle, Jennifer Jamieson, Anna Kalff, and Julian Bosco.
• Department of Intensive Care Medicine, The Royal Adelaide Hospital, Port Road, Adelaide, SA 5000, Australia; School of Public Health and Preventative Medicine, Monash University, Melbourne, VIC, Australia; Discipline of Acute Care Medicine, School of Medicine, The University of Adelaide, Adelaide, SA, Australia. Electronic address: eamon.raith@sa.gov.au.
• Heart Lung. 2018 Jul 1; 47 (4): 425-428.

BackgroundIdiopathic capillary leak syndrome (Clarkson's Disease) is a rare angiopathy with a heterogenous phenotype that may present as distributive shock refractory to resuscitative management.ObjectiveWe report a case of idiopathic systemic capillary leak syndrome presenting as septic shock.MethodsStructured case report and review of the literature.ResultsA 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.ConclusionsIdiopathic 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.Copyright © 2018 Elsevier Inc. All rights reserved.

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