The acute respiratory distress syndrome in catastrophic antiphospholipid syndrome:: analysis of a series of 47 patients
The acute respiratory distress syndrome in catastrophic antiphospholipid syndrome:: analysis of a series of 47 patients
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DOI：
10.1136/ard.2005.037671
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发表时间：
2006-01-01
影响因子：
27.4
通讯作者：
Font, J
中科院分区：
文献类型：
--
作者：
Bucciarelli, S;Espinosa, G;Font, J
背景：急性呼吸窘迫综合征（ARDS）是一种非心源性肺水肿，其特征是氧疗难以治愈的严重低氧血症，胸片显示弥漫性肺部浸润。各种严重的内科和外科疾病，包括全身性自身免疫性疾病，都可能引发这种疾病。抗磷脂综合征 (APS) 的“灾难性”变体是这种全身性自身免疫性疾病的加速形式，由于多处小血管闭塞，导致多器官衰竭。目的：分析发生 ARDS 的灾难性 APS 患者的临床和实验室特征。方法：ARDS 病例选自灾难性 APS 患者国际登记网站（CAPS 登记处） (http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM) 及其特征检查。结果：220 名灾难性 APS 患者中，有 150 名 (68%) 报告肺部受累，47 名患者 (21%) 被诊断为 ARDS。其中 19 名 (40%) 患者死亡。对 10 名患者进行了病理学研究，其中 7 名患者出现血栓性微血管病。伴有或不伴有ARDS的灾难性APS患者在年龄、性别、诱发因素、临床表现或死亡率方面没有差异。结论：ARDS是灾难性APS的主要肺部表现。因此，APS 背景下 ARDS 的存在使得有必要排除该综合征的灾难性变体的存在。
Background: The acute respiratory distress syndrome ( ARDS) is a non-cardiogenic form of pulmonary oedema characterised by severe hypoxaemia refractory to oxygen therapy, with diffuse pulmonary infiltrates on chest radiographs. It can be precipitated by various serious medical and surgical conditions, including systemic autoimmune diseases. The ''catastrophic'' variant of the antiphospholipid syndrome (APS) is an accelerated form of this systemic autoimmune condition which results in multiorgan failure because of multiple small vessel occlusions.Objective: To analyse the clinical and laboratory characteristics of patients with catastrophic APS who develop ARDS.Methods: Cases with ARDS were selected from the web site based international registry of patients with catastrophic APS (CAPS registry) (http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM) and their characteristics examined.Results: Pulmonary involvement was reported in 150 of 220 patients with catastrophic APS (68%) and 47 patients (21%) were diagnosed as having ARDS. Nineteen (40%) of these patients died. Pathological studies were undertaken in 10 patients and thrombotic microangiopathy was present in seven. There were no differences in age, sex, precipitating factors, clinical manifestations, or mortality between catastrophic APS patients with and without ARDS.Conclusions: ARDS is the dominant pulmonary manifestation of catastrophic APS. Thus the existence of ARDS in the context of an APS makes it necessary to rule out the presence of the catastrophic variant of this syndrome.