Nishiyama Osamu, Tohda Yuji
Department of Respiratory Medicine and Allergology, Kindai University, Faculty of Medicine.
Nihon Rinsho Meneki Gakkai Kaishi. 2017;40(3):139-144. doi: 10.2177/jsci.40.139.
Some patients with idiopathic interstitial pneumonias (IIPs) have clinical features that suggest autoimmune process but not fulfill established criteria for connective tissue disease (CTD). Different terms and criteria have been proposed to describe these patients, which includes undifferentiated connective tissue disease (UCTD), lung dominant connective tissue disease (LD-CTD), and autoimmune-featured interstitial lung disease (AIF-ILD). To marshal these ununiform classification criteria, the "European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease" proposed a consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity with the term "interstitial pneumonia with autoimmune features" (IPAF). It was reported that patients with IPAF accounted for 7.3%∼34.1% of patients with interstitial lung disease (ILD). Survival, rate of progressing to CTD, appropriate treatment, and treatment response are not yet elucidated. Furthermore, it is not determined whether IPAF is a distinct category of ILD or simply a part of IIP. These questions should be elucidated by future prospective cohort studies. Hopefully, rheumatologists should participate in not only clinical trials but also in multidisciplinary discussions for IPAF.
一些特发性间质性肺炎（IIP）患者具有提示自身免疫过程的临床特征，但不符合结缔组织病（CTD）的既定标准。已经提出了不同的术语和标准来描述这些患者，其中包括未分化结缔组织病（UCTD）、肺部为主的结缔组织病（LD-CTD）和具有自身免疫特征的间质性肺病（AIF-ILD）。为了整理这些不统一的分类标准，“欧洲呼吸学会/美国胸科学会未分化形式的结缔组织病相关间质性肺病特别工作组”就IIP患者以及具有自身免疫特征的患者的命名和分类标准达成了共识，术语为“具有自身免疫特征的间质性肺炎”（IPAF）。据报道，IPAF患者占间质性肺病（ILD）患者的7.3%至34.1%。生存率、进展为CTD的比率、适当的治疗方法以及治疗反应尚未阐明。此外，尚未确定IPAF是ILD的一个独特类别还是仅仅是IIP的一部分。这些问题应由未来的前瞻性队列研究来阐明。希望风湿病学家不仅应参与IPAF的临床试验，还应参与多学科讨论。
