Idiopathic Systemic Capillary Leak Syndrome: A Diagnostic Challenge and Its ManagementCorrespondence Address :
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.
Hypovolemic shock, Theophylline, Steroids
Janak Tarun Bahirwani, Raja Naga Mahesh Maddala, Weena Stanley, Mukhyaprana Prabhu. IDIOPATHIC SYSTEMIC CAPILLARY LEAK SYNDROME: A DIAGNOSTIC CHALLENGE
AND ITS MANAGEMENT. Journal of Clinical and Diagnostic Research [serial online] 2017 October [cited: 2018 Jan 21 ]; 11:OD01-OD02. Available from
Date of Submission: Jul 05, 2017
Date of Peer Review: Aug 04, 2017
Date of Acceptance: Aug 24, 2017
Date of Publishing: Oct 01, 2017
FINANCIAL OR OTHER COMPETING INTERESTS: None.
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