Jiang H D, Chen B
Department of Respiratory Medicine, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China.
Department of Respiratory and Critical Care Medicine, Affiliated Hospital of Xuzhou Medical University, Xuzhou 221000, China.
Zhonghua Yi Xue Za Zhi. 2021 Jun 1;101(20):1453-1457. doi: 10.3760/cma.j.cn112137-2020831-02514.
Interstitial lung disease (ILD), consisting of more than 200 subtypes of diseases, is a large group of heterogeneous diseases characterized by varying degrees of alveolitis and pulmonary parenchymal fibrosis. There are many challenges in its diagnosis and treatment. In recent years, new understanding of the clinical classification, disease behavior, early diagnosis, and treatment of ILD has been obtained. The anti-fibrotic drugs have been expanded successfully from treatment of idiopathic pulmonary fibrosis (IPF) to progressive-fibrosing interstitial lung diseases (PF-ILD). A new international clinical practice guideline categorized hypersensitivity pneumonitis (HP) into two clinical phenotypes, namely nonfibrotic and fibrotic HP. The clinical classification of ILD according to disease behavior and a progressing fibrotic phenotype is of important value for the establishment of treatment strategies for patients with ILD. Interstitial pulmonary abnormalities (ILA) may represent the subclinical stages of different types of ILD, and long-term follow-up of ILA is key to improving the early diagnosis of ILD. Interstitial pneumonia with autoimmune features (IPAF) may represent an early phase or prodromal state of a connective tissue disease (CTD), and patients with IPAF need to be under longitudinal surveillance for evolution to CTD.
间质性肺疾病（ILD）由200多种疾病亚型组成，是一大类异质性疾病，其特征为不同程度的肺泡炎和肺实质纤维化。其诊断和治疗存在诸多挑战。近年来，人们对ILD的临床分类、疾病行为、早期诊断和治疗有了新的认识。抗纤维化药物已成功从治疗特发性肺纤维化（IPF）扩展到进行性纤维化间质性肺疾病（PF-ILD）。一项新的国际临床实践指南将过敏性肺炎（HP）分为两种临床表型，即非纤维化型和纤维化型HP。根据疾病行为和进行性纤维化表型对ILD进行临床分类，对于制定ILD患者的治疗策略具有重要价值。间质性肺异常（ILA）可能代表不同类型ILD的亚临床阶段，对ILA进行长期随访是提高ILD早期诊断的关键。具有自身免疫特征的间质性肺炎（IPAF）可能代表结缔组织病（CTD）的早期阶段或前驱状态，IPAF患者需要接受长期监测，以观察是否演变为CTD。
