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结缔组织病相关间质性肺疾病:风湿病学家的观点

Connective tissue disease-associated interstitial lung disease: a rheumatologist's perspective.

作者信息

Connerton Áine, McCarthy Eoghan, Durcan Laura

机构信息

Beaumont Hospital, Dublin, Ireland.

出版信息

Breathe (Sheff). 2025 Jun 17;21(2):240166. doi: 10.1183/20734735.0166-2024. eCollection 2025 Apr.

Abstract

Pulmonary involvement is common in patients with underlying autoimmune and connective tissue diseases (CTDs) and can encompass a broad spectrum of disorders involving the airways, pleura, parenchyma, vascular system, bronchiectasis and nodules with significant overlap in these pathologies. Interstitial lung disease (ILD) is the most common pulmonary manifestation of systemic autoimmune rheumatic diseases and CTDs. Particular diseases, such as systemic sclerosis, rheumatoid arthritis and inflammatory myopathies, frequently associate with ILD and high-risk patients should be screened. Antibody profiling and imaging characteristics should be used to prognosticate where possible, along with regular surveillance to ensure therapies are optimised. Immunosuppressive therapies can be effective for patients with CTD-associated ILD, but difficulties arise in distinguishing between treatment failures and complications of immunosuppression.

摘要

肺部受累在患有自身免疫性和结缔组织疾病(CTD)的患者中很常见,可涵盖一系列广泛的疾病,包括气道、胸膜、实质、血管系统、支气管扩张和结节,这些病理情况有显著重叠。间质性肺病(ILD)是系统性自身免疫性风湿病和CTD最常见的肺部表现。某些特定疾病,如系统性硬化症、类风湿关节炎和炎性肌病,常与ILD相关,高危患者应进行筛查。应尽可能利用抗体谱分析和影像学特征进行预后评估,并定期监测以确保治疗得到优化。免疫抑制疗法对CTD相关ILD患者可能有效,但在区分治疗失败和免疫抑制并发症方面存在困难。

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