Kang Eun Ha, Song Yeong Wook
Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam 13620, Korea.
Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul 03080, Korea.
Pharmaceuticals (Basel). 2021 Mar 10;14(3):251. doi: 10.3390/ph14030251.
Among the diverse forms of lung involvement, interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are two important conditions in patients with rheumatic diseases that are associated with significant morbidity and mortality. The management of ILD and PAH is challenging because the current treatment often provides only limited patient survival benefits. Such challenges derive from their common pathogenic mechanisms, where not only the inflammatory processes of immune cells but also the fibrotic and proliferative processes of nonimmune cells play critical roles in disease progression, making immunosuppressive therapy less effective. Recently, updated treatment strategies adopting targeted agents have been introduced with promising results in clinical trials for ILD ad PAH. This review discusses the epidemiologic features of ILD and PAH among patients with rheumatic diseases (rheumatoid arthritis, myositis, and systemic sclerosis) and the state-of-the-art treatment options, focusing on targeted agents including biologics, antifibrotic agents, and vasodilatory drugs.
在肺部受累的多种形式中，间质性肺疾病（ILD）和肺动脉高压（PAH）是风湿性疾病患者的两种重要病症，它们与显著的发病率和死亡率相关。ILD和PAH的管理具有挑战性，因为目前的治疗通常仅能为患者提供有限的生存益处。这些挑战源于它们共同的致病机制，其中不仅免疫细胞的炎症过程，而且非免疫细胞的纤维化和增殖过程在疾病进展中都起着关键作用，这使得免疫抑制治疗效果较差。最近，采用靶向药物的更新治疗策略已被引入，在ILD和PAH的临床试验中取得了有前景的结果。本综述讨论了风湿性疾病（类风湿关节炎、肌炎和系统性硬化症）患者中ILD和PAH的流行病学特征以及最新的治疗选择，重点关注包括生物制剂、抗纤维化药物和血管扩张药物在内的靶向药物。
