Journal of Clinical and Diagnostic Research, ISSN - 0973 - 709X

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Case report
Table of Contents - Year : 2017 | Month : October | Volume : 11 | Issue : 10 | Page : OD01 - OD02

Idiopathic Systemic Capillary Leak Syndrome: A Diagnostic Challenge and Its Management OD01-OD02

Janak Tarun Bahirwani, Raja Naga Mahesh Maddala, Weena Stanley, Mukhyaprana Prabhu

Dr. Janak Tarun Bahirwani,
4, Jawahar Nagar, Prenderghast Road, Secunderabad-500003, Hyderabad, Telangana, India.

Idiopathic Systemic Capillary Leak Syndrome (ISCLS) is a fatal disorder characterised by recurrent episodes of hypotension, hypoalbuminemia and haemoconcentration. It is a rare disease, under-reported partly because of unawareness of treating physician. Here is a description of a 30 year old male presenting with history of fever, generalized oedema progressing to hypovolemic shock and multi organ dysfunction. His laboratory studies showed haemoconcentration, hypoalbuminemia and monoclonal gammopathy with negative bacteriological cultures. After excluding other probable etiologies he was diagnosed to have ISCLS. He was managed successfully with intravenous methylprednisolone, theophylline and other supportive measures. He has been put on prophylactic oral theophylline for one year.