Marc Pineton de Chambrun1, Charles-Edouard Luyt, François Beloncle, Marie Gousseff, Wladimir Mauhin, Laurent Argaud, Stanislas Ledochowski, Anne-Sophie Moreau, Romain Sonneville, Bruno Verdière, Sybille Merceron, Nathalie Zappella, Mickael Landais, Damien Contou, Alexandre Demoule, Sylvie Paulus, Bertrand Souweine, Bernard Lecomte, Antoine Vieillard-Baron, Nicolas Terzi, Elie Azoulay, Raymond Friolet, Marc Puidupin, Jérôme Devaquet, Jean-Marc Mazou, Yannick Fedun, Jean-Paul Mira, Jean-Herlé Raphalen, Alain Combes, Zahir Amoura. 1. 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.
Abstract
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.
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.
Authors: Simon Zec; Hisham A Mushtaq; Anwar Khedr; Abbas Bashir Jama; Ibtisam Rauf; Mikael Mir; Shikha Jain; Thoyaja Koritala; Juan P Domecq Garces; Brian Bartlett; Nitesh K Jain; Syed Anjum Khan Journal: J Community Hosp Intern Med Perspect Date: 2022-07-04
Authors: Christoph Schriefl; Christian Schoergenhofer; Florian Ettl; Michael Poppe; Christian Clodi; Matthias Mueller; Juergen Grafeneder; Bernd Jilma; Ingrid Anna Maria Magnet; Nina Buchtele; Magdalena Sophie Boegl; Michael Holzer; Fritz Sterz; Michael Schwameis Journal: Front Med (Lausanne) Date: 2021-06-09