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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

doi: 10.1097/CCM.0000000000002496
Clinical Investigations

Objective: 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.

Conclusions: 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.

Supplemental Digital Content is available in the text.

1Service de Médecine Interne 2, Centre Hospitalier Universitaire (CHU) La Pitié–Salpêtrière, Université Paris 6, APHP, Paris, France.

2Service de Réanimation Médicale, CHU La Pitié–Salpêtrière, Université Paris 6, APHP, Paris, France.

3Service de Réanimation Médicale, CHU d’Angers, Angers, France.

4Service de Médecine Interne, CH Bretagne Atlantique, Vannes, France.

5Service de Réanimation Médicale, CHU Edouard-Herriot, Lyon, France.

6Service de Réanimation, CHU Lyon-Sud, Pierre-Bénite, France.

7Service de Réanimation Polyvalente, CH Pierre Oudot, Bourgoin Jallieu, France.

8Service de Réanimation Médicale, CHRU Lille, Lille, France.

9Service de Réanimation Médicale, CHU Bichat, APHP, Paris, France.

10Service de Réanimation Polyvalente, Hôpital Delafontaine, Saint-Denis, France.

11Service de Réanimation Polyvalente, CH André-Mignot, Le Chesnay, France.

12Service de Réanimation Médicale, CHU Hôtel-Dieu, Nantes, France.

13Service de Réanimation Médicale, CHU Henri-Mondor, APHP, Créteil, France.

14Service de Pneumologie et de Réanimation Médicale, CHU La Pitié–Salpêtrière, APHP, Paris, France.

15Service de Réanimation Chirurgicale, CHU Lyon-Est, Hôpital Louis-Pradel, Bron, France.

16Service de Réanimation Médicale, Hôpital Gabriel-Montpied, CHU Clermont-Ferrand, Clermont-Ferrand, France.

17Service de Réanimation Polyvalente, CH Notre-Dame de la Miséricorde, Ajaccio, France.

18Service de Réanimation Médicale, CHU Ambroise-Paré, APHP, Boulogne-Billancourt, France.

19Service de Réanimation Médicale, CHU Grenoble, Grenoble, France.

20Service de Réanimation Médicale, CHU Saint-Louis, APHP, Paris, France.

21Service de Réanimation, Hôpital du Valais-Institut Central, Sion, Switzerland.

22Service de Réanimation, Hôpital d’Instruction des Armées Desgenettes, Lyon, France.

23Service de Réanimation Polyvalente, CH Foch, Suresnes, France.

24Service de Réanimation, CH Dax, Dax, France.

25Service de Réanimation Polyvalente, CH Bretagne Atlantique, Vannes, France.

26Service de Réanimation Médicale, CHU Cochin, APHP, Paris, France.

27Service de Réanimation Adulte, CHU Necker–Enfants Malades, APHP, Paris, France.

Members of the EurêClark Study Group are listed in Appendix 1 (Supplemental Digital Content 1,

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s website (

Dr. Luyt received funding from Bayer Healthcare and Merck Sharp and Dohme (MSD). Dr. Demoule’s institution received funding from Philips, and he received funding from Medtronic, MSD, Maquet, Covidien, Philips, Drager, Resmed, Fisher & Paykel. Dr. Azoulay’s institution received funding from MSD, Pfizer, Gilead, Fisher & Paykel, Alexion, and Basilea; he received funding from Gilead Sciences (board meeting) and funding for lectures from Astellas, Gilead, and Alexion; and he received support for article research from Assistance Publique - Hôpitaux de Paris France. The remaining authors have disclosed that they do not have any potential conflicts of interest.

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