Interstitial lung disease (ILD) encompasses a large group of pulmonary conditions sharing common clinical, radiological and histopathological features as a consequence of fibrosis of the lung interstitium. The majority of ILDs are idiopathic in nature with possible genetic predisposition, but is also well recognised as a complication of connective tissue disease or with certain environmental, occupational or drug exposures. In recent years, a concerted international effort has been made to standardise the diagnostic criteria in ILD subtypes, formalise multidisciplinary pathways and standardise treatment recommendations. In this review, we discuss some of the current challenges around ILD diagnostics, the role of serological testing, especially, in light of the new classification of Interstitial Pneumonia with Autoimmune Features (IPAF) and discuss the evidence for therapies targeted at idiopathic and immune-related pulmonary fibrosis.

中文翻译：

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特发性和免疫相关性肺纤维化：诊断和治疗挑战。

间质性肺疾病（ILD）涵盖了一大批肺部疾病，这些疾病由于肺间质纤维化而具有共同的临床，放射学和组织病理学特征。大多数ILDs具有特发性，可能具有遗传易感性，但也被公认是结缔组织病的并发症或某些环境，职业或药物暴露。近年来，国际上已经做出了一致的努力，以标准化ILD亚型的诊断标准，正规化多学科途径和标准化治疗建议。在这篇评论中，我们讨论了有关ILD诊断的当前挑战，血清学检测的作用，特别是