Interstitial lung disease unit, Royal Brompton Hospital, Fulham Road, Chelsea, London SW3 6NP, UK.
Centre for Rheumatology and Connective Tissue Disease, Division of Medicine, Royal Free Campus, University College London, London NW3 2QG, UK.
Nat Rev Rheumatol. 2014 Dec;10(12):728-39. doi: 10.1038/nrrheum.2014.149. Epub 2014 Sep 30.
Pulmonary complications are an important extra-articular feature of autoimmune rheumatic diseases and a major cause of mortality. The underlying pathogenesis probably involves multiple cellular compartments, including the epithelium, lung fibroblasts, and the innate and adaptive immune system. Heterogeneity in the extent and progression of lung fibrosis probably reflects differences in underlying pathogenic mechanisms. Growing understanding of the key pathogenic drivers of lung fibrosis might lead to the development of more effective targeted therapies to replicate the treatment advances in other aspects of these diseases. Interstitial lung disease (ILD) in connective tissue disease (CTD) is characterized using the classification of the idiopathic interstitial pneumonias. Systemic sclerosis is most frequently associated with ILD and, in most of these patients, ILD manifests as a histological pattern of nonspecific interstitial pneumonia. Conversely, in rheumatoid arthritis, the pattern of ILD is most often usual interstitial pneumonia. The key goals of clinical assessment of patients with both ILD and CTD are the detection of ILD and prognostic evaluation to determine which patients should be treated. Data from treatment trials in systemic sclerosis support the use of immunosuppressive therapy, with the treatment benefit largely relating to the prevention of progression of lung disease.
肺部并发症是自身免疫性风湿病的一个重要关节外特征，也是导致死亡率的一个主要原因。潜在的发病机制可能涉及多个细胞隔室，包括上皮细胞、肺成纤维细胞以及先天和适应性免疫系统。肺纤维化的程度和进展的异质性可能反映了潜在发病机制的差异。对肺纤维化关键发病驱动因素的深入了解可能会导致更有效的靶向治疗方法的发展，从而复制这些疾病其他方面的治疗进展。结缔组织病（CTD）中的间质性肺疾病（ILD）采用特发性间质性肺炎的分类进行描述。系统性硬化症与 ILD 的关联最为常见，在大多数此类患者中，ILD 表现为非特异性间质性肺炎的组织学模式。相反，在类风湿关节炎中，ILD 的模式通常为寻常间质性肺炎。ILD 和 CTD 患者临床评估的关键目标是检测 ILD 和预后评估，以确定哪些患者需要治疗。系统性硬化症治疗试验的数据支持使用免疫抑制疗法，其治疗益处主要与预防肺部疾病进展有关。
