Diagnosis of idiopathic pulmonary fibrosis (IPF) is based on defined clinical, radiographic, and pathological criteria.¹ IPF belongs to a heterogeneous group of interstitial lung diseases, and itself has varying presentations, prognosis, and responsiveness to drugs. The descriptor idiopathic was originally intended to separate IPF from other forms of pulmonary fibrosis with known causes, such as connective-tissue diseases, exposure to fibrogenic agents (eg, asbestos or drugs), and other disorders, such as sarcoidosis.

中文翻译：

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特发性肺纤维化：不再是特发性？

特发性肺纤维化 (IPF) 的诊断基于明确的临床、影像学和病理学标准。¹ IPF 属于一组异质性间质性肺疾病，其本身具有不同的表现、预后和对药物的反应性。特发性描述最初旨在将 IPF 与其他已知原因的肺纤维化形式区分开来，例如结缔组织疾病、暴露于纤维化剂（例如石棉或药物）和其他疾病，例如结节病。