Idiopathic Systemic Capillary Leak Syndrome: A Diagnostic Challenge
and Its Management
Published: October 1, 2017 | DOI: https://doi.org/10.7860/JCDR/2017/31384.10707
Janak Tarun Bahirwani, Raja Naga Mahesh Maddala, Weena Stanley, Mukhyaprana Prabhu
1. Undergraduate Student, Department of General Medicine, Kasturba Medical College, Manipal, Karnataka, India.
2. Senior Resident, Department of General Medicine, Kasturba Medical College, Manipal, Karnataka, India.
3. Associate Professor, Department of General Medicine, Kasturba Medical College, Manipal, Karnataka, India.
4. Professor, Department of General Medicine, Kasturba Medical College, Manipal, Karnataka, India.
Correspondence Address :
Dr. Janak Tarun Bahirwani,
4, Jawahar Nagar, Prenderghast Road, Secunderabad-500003, Hyderabad, Telangana, India.
E-mail: janaktb@gmail.com
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, 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.
Keywords
Hypovolemic shock, Theophylline, Steroids