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类风湿关节炎中严重闭塞性细支气管炎的特征描述

Characterisation of severe obliterative bronchiolitis in rheumatoid arthritis.

作者信息

Devouassoux G, Cottin V, Lioté H, Marchand E, Frachon I, Schuller A, Béjui-Thivolet F, Cordier J-F

机构信息

Hospices civils de Lyon, Centre Hospitalier Lyon Sud, Service de Pneumologie, Pierre-Bénite, Université de Lyon, France.

出版信息

Eur Respir J. 2009 May;33(5):1053-61. doi: 10.1183/09031936.00091608. Epub 2009 Jan 7.

DOI:10.1183/09031936.00091608
PMID:19129282
Abstract

The characteristics of patients with rheumatoid arthritis (RA) who develop obliterative bronchiolitis characterised by severe airflow obstruction have been hitherto poorly investigated. A retrospective study of 25 patients with RA and functional evidence of obliterative bronchiolitis (forced expiratory volume in one second (FEV(1))/forced vital capacity (FVC) <50% and/or residual volume (RV)/total lung capacity (TLC) >140% predicted) was conducted. Patients (mean+/-SD age 64+/-11 yrs) included 17 never-smokers and eight ex-smokers (10.5+/-5.4 pack-yrs). The diagnosis of RA preceded respiratory symptoms in 88% of cases. Dyspnoea on exertion was present in all patients and bronchorrhea in 44%. High-resolution computed tomography findings included: bronchial wall thickening (96%), bronchiectasis (40%), mosaic pattern (40%), centrilobular emphysema (56%), and reticular and/or ground-glass opacities (32%). Pulmonary function tests showed: FEV(1) 41+/-12% pred, FEV(1)/FVC 49+/-14%, FVC 70+/-20% pred, RV 148+/-68% pred and RV/TLC 142+/-34% pred. Lung biopsy, available in nine patients, demonstrated constrictive, follicular and mixed bronchiolitis. Patients were followed for 48.2+/-49 months. Treatment was poorly effective. Chronic respiratory failure occurred in 40% of patients, and four patients died. Obliterative bronchiolitis associated with rheumatoid arthritis is a severe and under-recognised condition leading to respiratory failure and death in a high proportion of patients.

摘要

类风湿关节炎(RA)患者发生以严重气流阻塞为特征的闭塞性细支气管炎的特点迄今研究甚少。对25例有闭塞性细支气管炎功能证据(一秒用力呼气容积(FEV₁)/用力肺活量(FVC)<50%和/或残气量(RV)/肺总量(TLC)>预测值的140%)的RA患者进行了一项回顾性研究。患者(平均±标准差年龄64±11岁)包括17例从不吸烟者和8例既往吸烟者(10.5±5.4包年)。88%的病例中RA诊断先于呼吸道症状出现。所有患者均有劳力性呼吸困难,44%有支气管分泌增多。高分辨率计算机断层扫描结果包括:支气管壁增厚(96%)、支气管扩张(40%)、马赛克样改变(40%)、小叶中心型肺气肿(56%)以及网状和/或磨玻璃样阴影(32%)。肺功能检查显示:FEV₁为预测值的41±l2%,FEV₁/FVC为49±14%,FVC为预测值的70±20%,RV为预测值的148±68%,RV/TLC为预测值的142±34%。9例患者进行了肺活检,显示为缩窄性、滤泡性和混合性细支气管炎。患者随访48.2±49个月。治疗效果不佳。40%的患者发生慢性呼吸衰竭,4例患者死亡。与类风湿关节炎相关的闭塞性细支气管炎是一种严重且未得到充分认识的疾病,导致高比例患者发生呼吸衰竭和死亡。

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