Pineton de Chambrun Marc, Luyt Charles-Edouard, Beloncle François, Gousseff Marie, Mauhin Wladimir, Argaud Laurent, Ledochowski Stanislas, Moreau Anne-Sophie, Sonneville Romain, Verdière Bruno, Merceron Sybille, Zappella Nathalie, Landais Mickael, Contou Damien, Demoule Alexandre, Paulus Sylvie, Souweine Bertrand, Lecomte Bernard, Vieillard-Baron Antoine, Terzi Nicolas, Azoulay Elie, Friolet Raymond, Puidupin Marc, Devaquet Jérôme, Mazou Jean-Marc, Fedun Yannick, Mira Jean-Paul, Raphalen Jean-Herlé, Combes Alain, Amoura Zahir
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.
Crit Care Med. 2017 Jul;45(7):1216-1223. 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.
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.
系统性毛细血管渗漏综合征是反复发生低血容量性休克的一种非常罕见的病因。关于其临床表现、实验室检查结果以及需要入住重症监护病房（ICU）的患者的预后，相关数据较少。本研究旨在描述严重系统性毛细血管渗漏综合征发作的临床表现及ICU管理情况。
设计、地点、患者：这项多中心回顾性分析涉及纳入欧洲克拉克森病（EurêClark）登记处且于1992年5月至2016年2月期间入住ICU的患者。
纳入了37例患者发生的59次发作（男女比例为1.05；平均±标准差年龄为51±11.4岁）。在34例（91.9%）患有单克隆免疫球蛋白G 型丙种球蛋白病的患者中，20例（58.8%）有κ轻链。入住ICU时的血红蛋白和蛋白血症中位数（四分位间距）分别为20.2 g/dL（17.9 - 22 g/dL）和50 g/L（36.5 - 58.5 g/L）。15次发作（25.4%）期间输注了静脉注射免疫球蛋白（IV免疫球蛋白）。12次发作（20.3%）期间出现了骨筋膜室综合征。11例（18.6%）患者在ICU死亡。二元分析（37例患者的最后一次发作）确定序贯器官衰竭评估评分大于10（比值比，12.9 [95%置信区间，1.2 - 140]；p = 0.04）和累积液体治疗量大于10.7 L（比值比，16.8 [1.6 - 180]；p = 0.02）为医院死亡率的独立预测因素。
我们描述了需要入住ICU的严重系统性毛细血管渗漏综合征发作的最大队列。大量液体治疗与较差的预后独立相关。使用IV免疫球蛋白与生存率改善无关；因此，应谨慎考虑其使用，并且在未来研究中需要进一步评估。
