Roschmann R A, Rothenberg R J
Semin Arthritis Rheum. 1987 Feb;16(3):174-85. doi: 10.1016/0049-0172(87)90020-5.
During the past four decades there has been a growing appreciation of the frequency of pulmonary abnormalities associated with RA. Approximately 30% to 40% of patients with RA demonstrate either radiographic or pulmonary function abnormalities indicative of interstitial fibrosis or restrictive lung disease. The severity of pulmonary fibrosis is not associated with rheumatologic symptoms or the duration of the associated RA, nor is there any clear relation to the extraarticular features of RA or serologic findings. Survival rates in patients with coexisting RA and pulmonary fibrosis are similar to those of patients with idiopathic pulmonary fibrosis. However, the spectrum of disease activity is quite variable. The majority of patients with progressive pulmonary symptomatology, when treated with corticosteroids, will have equivocal results. Some patients appear to respond to immunosuppressive or cytotoxic medications. The role of macrophages may be central to the injury to lung. Recent studies suggest a potential treatment role for cyclosporine, which may be able to interrupt lymphocyte-stimulated macrophage activation, and thus, fibroblast-mediated fibrosis in patients with pulmonary interstitial fibrosis. Bronchoalveolar lavage studies may delineate subgroups of patients who are more likely to respond to immunosuppressive agents, especially when treatment is started early.
在过去的四十年里，人们越来越认识到与类风湿关节炎（RA）相关的肺部异常的发生率。大约30%至40%的RA患者表现出影像学或肺功能异常，提示间质性纤维化或限制性肺病。肺纤维化的严重程度与风湿症状或相关RA的病程无关，也与RA的关节外表现或血清学结果没有明确关系。合并RA和肺纤维化患者的生存率与特发性肺纤维化患者相似。然而，疾病活动谱差异很大。大多数有进行性肺部症状的患者接受皮质类固醇治疗时，效果不明确。一些患者似乎对免疫抑制或细胞毒性药物有反应。巨噬细胞的作用可能是肺部损伤的关键。最近的研究表明环孢素可能具有潜在的治疗作用，它可能能够阻断淋巴细胞刺激的巨噬细胞活化，从而阻断肺间质纤维化患者中由成纤维细胞介导的纤维化。支气管肺泡灌洗研究可能会区分出更可能对免疫抑制剂有反应的患者亚组，尤其是在早期开始治疗时。
