Idiopathic systemic capillary leak syndrome

Priya Patil; Kashmira Hajare; Jyoti Bansode

doi:10.4103/ijccm.IJCCM_464_17

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Idiopathic systemic capillary leak syndrome

Priya Patil, Kashmira Hajare, Jyoti Bansode

Keywords : Agonist, beta-2 receptor, endothelial dysfunction, hemoconcentration, hypoalbuminemia, hypotension, idiopathic systemic capillary leak syndrome, theophylline

Citation Information : Patil P, Hajare K, Bansode J. Idiopathic systemic capillary leak syndrome. Indian J Crit Care Med 2018; 22 (5):369-371.

DOI: 10.4103/ijccm.IJCCM_464_17

License: CC BY-ND 3.0

Published Online: 01-05-2018

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Abstract

Idiopathic systemic capillary leak syndrome (ISCLS) is a very rare life-threatening disorder characterized by recurrent episodes of hypotension, hemoconcentration, and hypoalbuminemia. It is caused by transient endothelial dysfunction, leading to plasma leakage from intravascular to interstitial space. Here, we report a case of ISCLS with recurrent episodes of capillary leak which required a long-term prophylaxis with beta-2 adrenergic receptor agonist and theophylline. Although ISCLS is the rare entity, associated morbidity and mortality require physician\'s awareness to provide timely therapy. Under-recognition in the medical community and rarity of this syndrome has precluded analysis in rational clinical trial designs that are necessary to determine targeted and adequate therapy. This report is meant to enhance awareness of ISCLS among physician\'s community.

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Clarkson B, Thompson D, Horwith M, Luckey EH. Cyclical edema and shock due to increased capillary permeability. Am J Med 1960;29:193-216.
Druey KM, Greipp PR. Narrative review: The systemic capillary leak syndrome. Ann Intern Med 2010;153:90-8.
Kapoor P, Greipp PT, Schaefer EW, Mandrekar SJ, Kamal AH, Gonzalez-Paz NC, et al. Idiopathic systemic capillary leak syndrome (Clarkson's disease): The mayo clinic experience. Mayo Clin Proc 2010;85:905-12.
Gousseff M, Arnaud L, Lambert M, Hot A, Hamidou M, Duhaut P, et al. The systemic capillary leak syndrome: A case series of 28 patients from a European registry. Ann Intern Med 2011;154:464-71.
Lambert M, Launay D, Hachulla E, Morell-Dubois S, Soland V, Queyrel V, et al. High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome. Crit Care Med 2008;36:2184-7.
Assaly R, Olson D, Hammersley J, Fan PS, Liu J, Shapiro JI, et al. Initial evidence of endothelial cell apoptosis as a mechanism of systemic capillary leak syndrome. Chest 2001;120:1301-8.
Casnocha SA, Eskin SG, Hall ER, McIntire LV. Permeability of human endothelial monolayers: Effect of vasoactive agonists and cAMP. J Appl Physiol (1985) 1989;67:1997-2005.
Shimura H, Yamaguchi M, Kuzume M, Matsumiya A, Matsumoto T, Sakai H, et al. Prevention of reactive oxygen-induced endothelial cell injury by blocking its process. Eur Surg Res 1999;31:390-8.
Dhir V, Arya V, Malav IC, Suryanarayanan BS, Gupta R, Dey AB, et al. Idiopathic systemic capillary leak syndrome (SCLS): Case report and systematic review of cases reported in the last 16 years. Intern Med 2007;46:899-904.

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