Chronic idiopathic systemic capillary leak syndrome: a case report.

Autor: Alkhunaizi AM; 1Nephrology Section, Internal Medicine Services Division, Johns Hopkins Aramco Healthcare, Saudi Aramco, Box 10955, Dhahran, 31311 Saudi Arabia., Kabbani AH; 3Faculty of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia., ElTigani MA; 2General Internal Medicine, Johns Hopkins Aramco Healthcare, Dhahran, Saudi Arabia.
Jazyk: angličtina
Zdroj: Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology [Allergy Asthma Clin Immunol] 2019 May 28; Vol. 15, pp. 34. Date of Electronic Publication: 2019 May 28 (Print Publication: 2019).
DOI: 10.1186/s13223-019-0347-0
Abstrakt: Background: Idiopathic systemic capillary leak syndrome (ISCLS), is a rare disorder characterized by recurrent attacks of varying severity of hypovolemic shock and generalized edema in association with hemoconcentration and hypoalbuminemia in the absence of albuminuria. The chronic form of ISCLS is extremely rare with only a few cases reported in the literature.
Case Presentation: Here we report the case of a young woman who developed chronic ISCLS characterized by massive, generalized and persistent edema. The work up confirmed the presence of monoclonal gammopathy. She was treated with several agents with no response.
Conclusion: Chronic ISCLS is a very rare disease of unknown etiology and results in devastating complications. The medical community should be aware of this disease with the hope that targeted therapy will become available in the future.
Competing Interests: Competing interestsThe authors declare that they no competing interests.
Databáze: MEDLINE
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