Rocky Vista University College of Osteopathic Medicine, Parker, Colorado.
Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, Italy.
Semin Respir Crit Care Med. 2019 Apr;40(2):271-277. doi: 10.1055/s-0039-1684007. Epub 2019 May 28.
In 2015, a multidisciplinary task force comprising pulmonologists, rheumatologists, pathologists, and radiologists representing the European Respiratory Society and American Thoracic Society published a diagnostic classification schema for individuals with interstitial lung disease and autoimmune features who did not meet criteria for a defined connective tissue disease. The term interstitial pneumonia with autoimmune features (IPAF) was applied. Classification criteria are often nonspecific, but up to 90% of subjects with IPAF have serological evidence for autoimmunity (particularly (+) antinuclear antibodies). Distinguishing patients with IPAF from idiopathic pulmonary disorders may be difficult. The natural history and appropriate management of IPAF have not been clarified, as data are largely limited to retrospective studies. In this review, we discuss the salient clinical, serologic, histologic, and radiographic features of IPAF and discuss an approach to management.
2015 年，一个由肺科医生、风湿病学家、病理学家和放射科医生组成的多学科工作组代表欧洲呼吸学会和美国胸科学会发表了一个诊断分类方案，用于那些有间质性肺疾病和自身免疫特征但不符合特定结缔组织疾病标准的患者。术语“具有自身免疫特征的间质性肺炎（IPAF）”被应用。分类标准通常是非特异性的，但高达 90%的 IPAF 患者有自身免疫的血清学证据（特别是（+）抗核抗体）。区分 IPAF 患者和特发性肺部疾病可能很困难。IPA 的自然病史和适当的管理尚未阐明，因为数据主要限于回顾性研究。在这篇综述中，我们讨论了 IPAF 的显著临床、血清学、组织学和影像学特征，并讨论了管理方法。
