Idiopathic systemic capillary leak syndrome

A diagnostic challenge and its management

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

Research output: Contribution to journalArticle

Abstract

Idiopathic Systemic Capillary Leak Syndrome (ISCLS) is a fatal disorder characterised by recurrent episodes of hypotension, hypoalbuminemia and haemoconcentration. It is a rare disease, underreported 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 haemoconcenration, 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.

Original languageEnglish
Pages (from-to)OD01-OD02
JournalJournal of Clinical and Diagnostic Research
Volume11
Issue number10
DOIs
Publication statusPublished - 01-10-2017

Fingerprint

Capillary Leak Syndrome
Hypoalbuminemia
Theophylline
Paraproteinemias
Methylprednisolone
Rare Diseases
Hypotension
Shock
Edema
Fever
Physicians

All Science Journal Classification (ASJC) codes

  • Clinical Biochemistry

Cite this

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Idiopathic systemic capillary leak syndrome : A diagnostic challenge and its management. / Bahirwani, Janak Tarun; Maddala, Raja Naga Mahesh; Stanley, Weena; Prabhu, Mukhyaprana.

In: Journal of Clinical and Diagnostic Research, Vol. 11, No. 10, 01.10.2017, p. OD01-OD02.

Research output: Contribution to journalArticle

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