特发性肺纤维化(IPF)是一种病因不明的慢性间质性肺病(ILD)，该病在放射学和病理组织学上表现为普通型间质性肺炎，并伴有肺实质纤维化和过度胶原沉积[1,2]。

参考文献

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[2] Selman M, King TE, Pardo A. Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy[J]. Ann Intern Med, 2001, 134(2): 136-51.

[3] White ES, Lazar MH, Thannickal VJ. Pathogenetic mechanisms in usual interstitial pneumonia/idiopathic pulmonary fibrosis[J]. J Pathol, 2003, 201(3): 343-54.

[4] 程洁, 毛燕君, 鲁敏, 等. 特发性肺纤维化的非药物治疗进展[J]. 国际呼吸杂志, 2018, 38(15): 1194-1198.

[5] Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline[J]. Am J Respir Crit Care Med, 2022, 205(9): e18-e47.

[6] 叶俏, 代华平. 特发性肺纤维化诊断和治疗中国专家共识[J]. 中华结核和呼吸杂志, 2016, 39(06): 427-432.

来源：北京朝阳医院药事部