National Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, University of Lyon, INRAE, Lyon, France.
Expert Rev Respir Med. 2022 May;16(5):541-553. doi: 10.1080/17476348.2022.2089116. Epub 2022 Jun 29.
Rheumatoid arthritis (RA) is the most common inflammatory autoimmune disease, characterized by symmetric destructive arthritis and synovitis. Lung involvement is frequent, including in the form of interstitial lung disease (ILD). RA-ILD often presents with a radiologic and pathologic pattern of usual interstitial pneumonia, similar to idiopathic pulmonary fibrosis, highlighting the similarities between the two diseases, but other patterns and pathological associations are described.
This article reviews the pathogenesis of pulmonary fibrosis in the setting of rheumatoid arthritis as well as the current and future therapeutic options.
Pulmonary fibrosis in the setting of RA-ILD is an example of genotype-environment interaction and involves multiple mechanisms including autoimmunity, inflammation, and fibrogenesis. Although ILD conveys most of the exceeding mortality in RA patients, there are no official guidelines for the management of RA-ILD. Attention should be paid to potential lung toxicity of RA treatment even though some of them might help stabilize the ILD. Current standard of care is often composed of glucocorticoids that may be associated with immunosuppressive therapy. Following the approval of antifibrotic therapy for ILDs with a progressive fibrosing phenotype, current works are evaluating the benefit of such treatment in RA-ILD.
类风湿关节炎(RA)是最常见的炎症性自身免疫性疾病,其特征为对称性破坏性关节炎和滑膜炎。肺部受累很常见,包括间质性肺疾病(ILD)。RA-ILD 常表现为特发性肺纤维化相似的普通间质性肺炎的放射学和病理学模式,突出了两种疾病之间的相似性,但也描述了其他模式和病理关联。
本文综述了类风湿关节炎中肺纤维化的发病机制以及目前和未来的治疗选择。
RA-ILD 中的肺纤维化是基因型-环境相互作用的一个例子,涉及多种机制,包括自身免疫、炎症和纤维化。虽然ILD 导致 RA 患者的大部分超额死亡率,但目前还没有针对 RA-ILD 管理的官方指南。即使其中一些可能有助于稳定ILD,也应注意 RA 治疗的潜在肺部毒性。目前的标准治疗通常包括可能与免疫抑制治疗相关的糖皮质激素。在具有进行性纤维化表型的 ILD 的抗纤维化治疗获得批准后,目前的研究正在评估此类治疗在 RA-ILD 中的获益。