McLean-Tooke Andrew, Moore Irene, Lake Fiona
Department of Clinical Immunology Sir Charles Gairdner Hospital Perth WA Australia.
Department of Laboratory Immunology PathWest QEII Medical Centre Perth WA Australia.
Clin Transl Immunology. 2019 Nov 5;8(11):e1086. doi: 10.1002/cti2.1086. eCollection 2019.
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.
间质性肺疾病（ILD）包括一大类肺部疾病，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ILD本质上是特发性的，可能存在遗传易感性，但它也被公认为是结缔组织病的并发症，或与某些环境、职业或药物暴露有关。近年来，国际上齐心协力对ILD亚型的诊断标准进行了标准化，规范了多学科诊疗路径，并对治疗建议进行了标准化。在本综述中，我们讨论了当前ILD诊断面临的一些挑战，血清学检测的作用，特别是鉴于自身免疫特征性间质性肺炎（IPAF）的新分类，并讨论了针对特发性和免疫相关性肺纤维化的治疗证据。
