与类风湿关节炎相关的闭塞性细支气管炎：单中心病例系列分析。
Obliterative bronchiolitis associated with rheumatoid arthritis: analysis of a single-center case series.
机构信息
Department of Internal Medicine, 200 First St. SW, Rochester, MN, 55905, USA.
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN, 55905, USA.
出版信息
BMC Pulm Med. 2018 Jun 22;18(1):105. doi: 10.1186/s12890-018-0673-x.
BACKGROUND
Rheumatoid arthritis (RA) is a systemic autoimmune condition characterized by erosive inflammation of the joints. One rare pulmonary manifestation is obliterative bronchiolitis (OB), a small airways disease characterized by the destruction of bronchiolar epithelium and airflow obstruction.
METHODS
We retrospectively reviewed the clinical data of patients with rheumatoid arthritis-associated obliterative bronchiolitis (RA-OB) from 01/01/2000 to 12/31/2015. Presenting clinical features, longitudinal pulmonary function testing, radiologic findings, and independent predictors of all-cause mortality were assessed.
RESULTS
Forty one patients fulfilled criteria for diagnosis of RA-OB. There was notable female predominance (92.7%) with a mean age of 57 ± 15 years. Dyspnea was the most common presenting clinical symptom. Median FEV1 was 40% (IQR 31-52.5) at presentation, with a mean decline of - 1.5% over a follow-up period of thirty-three months. Associated radiologic findings included mosaic attenuation and pulmonary nodules. A majority of patients (78%) received directed therapy including long-acting inhalers, systemic corticosteroids or other immunosuppressive agents, and macrolide antibiotics. All-cause mortality was 27% over a median follow-up of sixty-two months (IQR 32-113). No distinguishable predictors of survival at presentation were found.
CONCLUSIONS
RA-OB appears to have a stable clinical course in the majority of patients despite persistent symptoms and severe obstruction based on presenting FEV1.
背景
类风湿关节炎（RA）是一种以关节侵蚀性炎症为特征的系统性自身免疫性疾病。一种罕见的肺部表现是闭塞性细支气管炎（OB），这是一种小气道疾病，其特征是细支气管上皮破坏和气流阻塞。
方法
我们回顾性地分析了 2000 年 1 月 1 日至 2015 年 12 月 31 日期间与类风湿关节炎相关的闭塞性细支气管炎（RA-OB）患者的临床资料。评估了患者的临床表现、纵向肺功能检测、影像学发现和全因死亡率的独立预测因素。
结果
41 例患者符合 RA-OB 的诊断标准。患者以女性为主（92.7%），平均年龄为 57±15 岁。呼吸困难是最常见的临床表现。FEV1 的中位数（IQR 31-52.5）在就诊时为 40%，随访 33 个月期间平均下降了-1.5%。相关的影像学发现包括马赛克衰减和肺结节。大多数患者（78%）接受了靶向治疗，包括长效吸入剂、全身皮质激素或其他免疫抑制剂和大环内酯类抗生素。中位随访 62 个月（IQR 32-113）时，全因死亡率为 27%。在就诊时未发现可识别的生存预测因素。
结论
尽管基于就诊时的 FEV1 存在持续的症状和严重的阻塞，RA-OB 似乎在大多数患者中具有稳定的临床过程。
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