Kang Hyung Koo, Song Jin Woo
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Inje University Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Republic of Korea.
Department of Pulmonology and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Tuberc Respir Dis (Seoul). 2024 Apr;87(2):123-133. doi: 10.4046/trd.2023.0119. Epub 2023 Dec 18.
Interstitial lung diseases (ILDs) are a diverse collection of lung disorders sharing similar features, such as inflammation and fibrosis. The diagnosis and management of ILD require a multidisciplinary approach using clinical, radiological, and pathological evaluation. Progressive pulmonary fibrosis (PPF) is a distinct form of progressive and fibrotic disease, occurring in ILD cases other than in idiopathic pulmonary fibrosis (IPF). It is defined based on clinical symptoms, lung function, and chest imaging, regardless of the underlying condition. The progression to PPF must be monitored through a combination of pulmonary function tests (forced vital capacity [FVC] and diffusing capacity of the lung for carbon monoxide), an assessment of symptoms, and computed tomography scans, with regular follow-up. Although the precise mechanisms of PPF remain unclear, there is evidence of shared pathogenetic mechanisms with IPF, contributing to similar disease behavior and worse prognosis compared to non-PPF ILD. Pharmacological treatment of PPF includes immunomodulatory agents to reduce inflammation and the use of antifibrotics to target progressive fibrosis. Nintedanib, a known antifibrotic agent, was found to be effective in slowing IPF progression and reducing the annual rate of decline in FVC among patients with PPF compared to placebos. Nonpharmacological treatment, including pulmonary rehabilitation, supplemental oxygen therapy, and vaccination, also play important roles in the management of PPF, leading to comprehensive care for patients with ILD. Although there is currently no cure for PPF, there are treatments that can help slow the progression of the disease and improve quality of life.
间质性肺疾病（ILDs）是一组具有相似特征（如炎症和纤维化）的多种肺部疾病。ILD的诊断和管理需要采用多学科方法，包括临床、放射学和病理学评估。进行性肺纤维化（PPF）是一种独特的进行性纤维化疾病形式，发生在特发性肺纤维化（IPF）以外的ILD病例中。它是根据临床症状、肺功能和胸部影像学来定义的，而不考虑潜在病因。必须通过肺功能测试（用力肺活量[FVC]和肺一氧化碳弥散量）、症状评估和计算机断层扫描相结合，并定期随访，来监测向PPF的进展情况。虽然PPF的确切机制尚不清楚，但有证据表明其与IPF存在共同的发病机制，与非PPF的ILD相比，导致了相似的疾病行为和更差的预后。PPF的药物治疗包括使用免疫调节剂来减轻炎症以及使用抗纤维化药物来针对进行性纤维化。已知的抗纤维化药物尼达尼布被发现与安慰剂相比，在减缓PPF患者的IPF进展和降低FVC年下降率方面有效。非药物治疗，包括肺康复、补充氧气疗法和疫苗接种，在PPF的管理中也发挥着重要作用，从而为ILD患者提供全面护理。虽然目前PPF无法治愈，但有一些治疗方法可以帮助减缓疾病进展并提高生活质量。
