Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Ther Adv Respir Dis. 2024 Jan-Dec;18:17534666241288417. doi: 10.1177/17534666241288417.
Fibrosing interstitial lung diseases (FILDs) other than idiopathic pulmonary fibrosis (IPF) can develop into progressive pulmonary fibrosis (PPF) despite initial management. A substantial proportion of patients with non-IPF interstitial lung diseases (ILDs) progress to PPF, including connective tissue disease-associated ILD (such as rheumatoid arthritis-associated ILD, systemic sclerosis-associated ILD, and idiopathic inflammatory myositis-associated ILD), fibrosing hypersensitivity pneumonitis, and fibrosing occupational ILD. The concept of PPF emerged only recently and several studies have confirmed the impact of PPF on mortality. In addition to poor prognosis among patients with PPF, there remains a lack of consensus in the diagnosis and treatment of PPF across different types of ILDs. There is a need to raise awareness of PPF in FILDs and to explore measures to improve PPF diagnosis and treatment, which in turn could potentially reduce the progression from FILD to PPF. This review discusses the disease burden of PPF and recent advances in the management of PPF among patients with ILDs, including antifibrotic medications that have emerged as promising treatment options. Additionally, this review highlights the perspectives of expert Chinese physicians with regard to their experience in managing PPF in clinical practice.
除特发性肺纤维化（IPF）外的纤维性间质性肺疾病（FILDs），尽管初始治疗，仍可进展为进行性肺纤维化（PPF）。相当一部分非特发性间质性肺疾病（ILDs）患者进展为 PPF，包括结缔组织病相关ILD（如类风湿关节炎相关ILD、系统性硬化症相关ILD 和特发性炎症性肌病相关ILD）、纤维性过敏性肺炎和纤维性职业性ILD。PPF 的概念最近才出现，几项研究已经证实了 PPF 对死亡率的影响。除了 PPF 患者预后不良外，不同类型的ILD 之间在 PPF 的诊断和治疗方面仍缺乏共识。需要提高对 FILDs 中 PPF 的认识，并探索改善 PPF 诊断和治疗的措施，这反过来可能减少从 FILD 向 PPF 的进展。这篇综述讨论了 PPF 的疾病负担以及 ILD 患者 PPF 管理方面的最新进展，包括作为有前途治疗选择的抗纤维化药物。此外，这篇综述还突出了中国专家医生在 PPF 临床管理方面的观点。
