We thank Marcel Koenigkam Santos and colleagues for their thoughtful comments on our Review.¹ We agree that interstitial lung disease (ILD) with airway-centered fibrosis represents a major diagnostic challenge, and that patients with this pattern often have hypersensitivity pneumonitis,² gastro-oesophageal reflux, aspiration, or connective tissue disease.³ Given these multiple associations, the prevalence of idiopathic airway-centered fibrosis⁴ remains unclear. The ATS/ERS classification paper in 2013 recognised bronchiolocentric patterns of interstitial pneumonia as a rare histological finding, but it was not clear whether this finding could be idiopathic.

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特发性肺纤维化的诊断标准–作者的回复

我们感谢Marcel Koenigkam Santos及其同事对我们的审查提出了深思熟虑的意见。¹我们同意以气道为中心的纤维化的间质性肺疾病（ILD）代表了主要的诊断挑战，并且具有这种模式的患者通常患有超敏性肺炎，²胃食管反流，误吸或结缔组织病。³考虑到这些多重关联，特发性以气道为中心的纤维化⁴的患病率仍不清楚。ATS / ERS分类论文在2013年将间质性肺炎的细支气管中心型识别为罕见的组织学发现，但尚不清楚该发现是否可能是特发性的。