Castelino Flavia V, Moua Teng
Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.
Mayo Clinic, Rochester, Minnesota.
ACR Open Rheumatol. 2021 May;3(5):295-304. doi: 10.1002/acr2.11253. Epub 2021 Mar 28.
Interstitial lung disease (ILD) is a common manifestation of connective tissue diseases (CTDs). A proportion of patients with CTD-ILDs develop progressive fibrosing ILD, which is characterized by worsening fibrotic abnormalities on high-resolution computed tomography scan, decline in lung function, worsening symptoms, and early mortality. Here, we review the impact of ILD in patients with CTDs, the importance of prompt diagnosis and close monitoring, and the evidence available to guide the management of CTD-ILDs. Management of patients with CTD-ILDs should be individualized and involve close collaboration between rheumatologists and pulmonologists. Immunosuppression is the mainstay of therapy for CTDs, but evidence for its effectiveness in slowing the progression of ILD is limited. Recently, nintedanib has been approved to slow decline in lung function in patients with systemic sclerosis-associated ILD and chronic fibrosing ILDs with a progressive phenotype. The results of ongoing clinical trials will help clinicians take a more evidence-based approach to the treatment of CTD-ILDs.
间质性肺疾病（ILD）是结缔组织病（CTD）的常见表现。一部分CTD-ILD患者会发展为进行性纤维化ILD，其特征是高分辨率计算机断层扫描显示纤维化异常加重、肺功能下降、症状恶化以及早期死亡。在此，我们综述ILD对CTD患者的影响、及时诊断和密切监测的重要性，以及可用于指导CTD-ILD管理的现有证据。CTD-ILD患者的管理应个体化，需要风湿科医生和肺科医生密切合作。免疫抑制是CTD治疗的主要手段，但关于其在减缓ILD进展方面有效性的证据有限。最近，尼达尼布已被批准用于减缓系统性硬化症相关ILD和具有进行性表型的慢性纤维化ILD患者的肺功能下降。正在进行的临床试验结果将有助于临床医生采用更基于证据的方法治疗CTD-ILD。
