自身免疫性间质性肺炎研究进展

许多间质性肺炎患者伴有自身免疫特征，但临床症状及血清学检查结果不能诊断为特定的结缔组织疾病类型。研究人员提出不同的命名和诊断标准来描述此类疾病，如未分化结缔组织疾病、肺优势结缔组织疾病或免疫特征的肺间质纤维化。由于没有统一的命名及诊断标准，限制了对这类疾病的研究。ERS 和 ATS “未分化结缔组织疾病与间质性肺疾病工作组”，将此类疾病命名为自身免疫性间质性肺炎(IPAF)。从三个方面：临床特点、血清学抗体、形态学包括肺部影像学及组织病理学，明确了诊断标准。此概念的提出，对此类疾病的研究搭建了一个新的统一的平台。

The research progress of interstitial pneumonia with autoimmune features

Many patients with an idiopathic interstitial pneumonia(IIP) have clinical features that suggest an underlying autoimmune process but do not meet established criteria for a connective tissue disease(CTD).Researchers have proposed differing criteria and terms to describe these patients,such as undifferentiated connective tissue disease (UCTD),lung-dominant connective tissue disease (Lung-dominant CTD),autoimmune-featured ILD,and lack of consensus over nomenclature and classification limits the ability to conduct prospective studies of a uniform cohort.The "European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease" was formed to create consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity.The task force proposes the term "interstitial pneumonia with autoimmune features"(IPAF) and offers classification criteria organised around the presence of a combination of features from three domains:a clinical domain consisting of specific extra-thoracic features,a serologic domain consisting of specific autoantibodies,and a morphologic domain consisting of specific chest imaging,histopathologic or pulmonary physiologic features.With IPAF,a sound platform has been provided from which to launch the requisite future research investigations of a more uniform cohort.