具有自身免疫特征的间质性肺炎的临床特点及转归

目的分析具有自身免疫疾病特征的间质性肺炎（IPAF）的临床特点及与自身免疫性疾病之间的相互联系，验证IPAF最新诊断标准的临床应用价值。方法回顾性分析2014年1月至2017年1月于我院住院的间质性肺疾病（ILD）患者，筛选其中符合（或部分符合）IPAF诊断标准的患者，详细记录患者临床表现、血清学检查结果及根据胸部高分辨CT (HRCT) 影像的分型结果，分析IPAF患者及部分符合IPAF诊断患者的临床特点，随访入组患者自身免疫性疾病相关的临床转归。结果研究共纳入患者90例，其中符合IPAF诊断38例，部分符合IPAF诊断52例，入组患者平均年龄（62.34±14.98）岁，IPAF患者间质性肺炎以非特异性间质性肺炎（NSIP）型为主要表现，部分符合IPAF诊断患者间质性肺炎以普通型间质性肺炎（UIP）型为主要表现。11例患者在随访中确诊自身免疫性疾病（其中7例为部分符合IPAF诊断患者，4例为IPAF患者）。Cox模型多因素分析提示是否完全满足IPAF诊断标准是疾病复发的影响因素。结论IPAF与自身免疫性疾病密切相关，部分IPAF患者（包括部分符合IPAF诊断患者）可能发展为系统性自身免疫性疾病；IPAF最新诊断标准为该类疾病的诊治提供了基础，但尚需更多临床研究对其补充、完善。

英文摘要:

Objective To analyze the clinical features of interstitial pneumonia with autoimmune features （IPAF） and the correlation between them. MethodsWe respectively analyzed the patients with interstitial lung disease （ILD） admitted in our hospital from January 2014 to January 2017. The patients who met all priori requirements and at least one feature of one domain were recuited,and the clinical features and autoimmune diseases related prognosis were analyzed. ResultsThere were 90 patients recruited, including 38 patients completely met IPAF classification criteria and 52 patients who incompletely met. The average age was (62.34±14.98) yr.. The pneumonia pattern of complete IPAF patients was non-specific interstitial pneumonia (NSIP), while it was usually interstitial pneumonia (UIP) in the incomplete IPAF patients. During follow-up, 11 patients were diagnosed with autoimmune diseases （4 with complete IPAF, and 7 with incomplete IPAF）. According to Cox regression analysis, completely meet the criteria of IPAF was related to the relapse of disease. ConclusionsThere exist relation between IPAF and autoimmune diseases. The patients with IPAFmay finally develop into autoimmune diseases. The IPAF classification criteria provide basic structure for this disease, but the limitation of the criteria call for revising by more clinical trials.

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