. Divisão de Pneumologia, Instituto do Coração - InCor - Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo (SP) Brasil.
J Bras Pneumol. 2023 Aug 7;49(4):e20230085. doi: 10.36416/1806-3756/e20230085. eCollection 2023.
Idiopathic pulmonary fibrosis (IPF) is a devastating chronic lung disease without a clear recognizable cause. IPF has been at the forefront of new diagnostic algorithms and treatment developments that led to a shift in patients' care in the past decade, indeed influencing the management of fibrotic interstitial lung diseases other than IPF itself. Clinical presentation, pathophysiology, and diagnostic criteria are briefly addressed in this review article. Additionally, evidence regarding the use of antifibrotics beyond the settings of clinical trials, impact of comorbidities, and therapeutic approaches other than pharmacological treatments are discussed in further detail.
特发性肺纤维化（IPF）是一种病因不明的严重慢性肺部疾病。在过去十年中，IPF 一直处于新的诊断算法和治疗方法的前沿，这确实改变了患者的治疗方式，甚至影响了除 IPF 本身之外的纤维性间质性肺疾病的管理。本文简要介绍了其临床表现、病理生理学和诊断标准。此外，还进一步详细讨论了临床试验以外的抗纤维化药物的使用、合并症的影响以及药物治疗以外的治疗方法的证据。
