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间质性肺疾病（ILD）是以不同程度肺泡炎和肺纤维化为病理特征的一大组异质性疾病，包括200多个病种，其诊断和治疗存在诸多挑战。近年来对于ILD的临床分类、疾病行为、早期诊断和治疗方面有了新的认识。抗纤维化药物从治疗特发性肺纤维化（IPF）成功拓展到进展性纤维化性ILD（PF-ILD）的治疗。新的国际临床指南将过敏性肺炎（HP）分为非纤维化性HP和纤维化性HP。根据疾病行为和致纤维化表型对ILD进行临床分类，对于治疗方案的制定具有重要指导价值。肺间质异常（ILA）可能代表了不同类型ILD的亚临床阶段，对其长期随访有助于ILD的早期诊断。具有自身免疫特征的间质性肺炎（IPAF）或许是结缔组织病（CTD）的早期阶段或前驱状态，长期动态监测其向CTD的演变至关重要。“,”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.