The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission : Critical Care Medicine

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The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission

Pineton de Chambrun, Marc MD, MSc1,2; Luyt, Charles-Edouard MD, PhD2; Beloncle, François MD, MSc3; Gousseff, Marie MD4; Mauhin, Wladimir MD, MSc1; Argaud, Laurent MD, PhD5; Ledochowski, Stanislas MD6,7; Moreau, Anne-Sophie MD8; Sonneville, Romain MD, PhD9; Verdière, Bruno MD10; Merceron, Sybille MD11; Zappella, Nathalie MD11; Landais, Mickael MD12; Contou, Damien MD13; Demoule, Alexandre MD, PhD14; Paulus, Sylvie MD15; Souweine, Bertrand MD, PhD16; Lecomte, Bernard MD17; Vieillard-Baron, Antoine MD, PhD18; Terzi, Nicolas MD, PhD19; Azoulay, Elie MD, PhD20; Friolet, Raymond MD21; Puidupin, Marc MD22; Devaquet, Jérôme MD23; Mazou, Jean-Marc MD24; Fedun, Yannick MD25; Mira, Jean-Paul MD, PhD26; Raphalen, Jean-Herlé MD27; Combes, Alain MD, PhD2; Amoura, Zahir MD, MSc1

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Critical Care Medicine 45(7):p 1216-1223, July 2017. | DOI: 10.1097/CCM.0000000000002496



Systemic capillary-leak syndrome is a very rare cause of recurrent hypovolemic shock. Few data are available on its clinical manifestations, laboratory findings, and outcomes of those patients requiring ICU admission. This study was undertaken to describe the clinical pictures and ICU management of severe systemic capillary-leak syndrome episodes.

Design, Setting, Patients: 

This multicenter retrospective analysis concerned patients entered in the European Clarkson’s disease (EurêClark) Registry and admitted to ICUs between May 1992 and February 2016.

Measurements and Main Results: 

Fifty-nine attacks occurring in 37 patients (male-to-female sex ratio, 1.05; mean ± sd age, 51 ± 11.4 yr) were included. Among 34 patients (91.9%) with monoclonal immunoglobulin G gammopathy, 20 (58.8%) had kappa light chains. ICU-admission hemoglobin and proteinemia were respectively median (interquartile range) 20.2 g/dL (17.9–22 g/dL) and 50 g/L (36.5–58.5 g/L). IV immunoglobulins were infused (IV immunoglobulin) during 15 episodes (25.4%). A compartment syndrome developed during 12 episodes (20.3%). Eleven (18.6%) in-ICU deaths occurred. Bivariable analyses (the 37 patients’ last episodes) retained Sequential Organ-Failure Assessment score greater than 10 (odds ratio, 12.9 [95% CI, 1.2–140]; p = 0.04) and cumulated fluid-therapy volume greater than 10.7 L (odds ratio, 16.8 [1.6–180]; p = 0.02) as independent predictors of hospital mortality.


We described the largest cohort of severe systemic capillary-leak syndrome flares requiring ICU admission. High-volume fluid therapy was independently associated with poorer outcomes. IV immunoglobulin use was not associated with improved survival; hence, their use should be considered prudently and needs further evaluation in future studies.

Copyright © 2017 by the Society of Critical Care Medicine and Wolters Kluwer Health, Inc. All Rights Reserved.

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