Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Respirology. 2020 Jun;25(6):636-643. doi: 10.1111/resp.13665. Epub 2019 Aug 6.
Idiopathic interstitial pneumonia (IIP) with autoimmune features that does not fulfil connective tissue disease (CTD) criteria has been recently defined as interstitial pneumonia with autoimmune features (IPAF). However, its long-term clinical course and outcome are poorly understood.
We included consecutive patients diagnosed with IIP (n = 586) or CTD-related interstitial lung disease (CTD-ILD, n = 149). Some patients with IIP were reclassified as IPAF based on recent guidelines.
The median follow-up period was 45 months. Among the IIP patients, 109 (18.6%) were reclassified as IPAF. Compared to the non-IPAF-IIP group, the IPAF group had slower diffusing capacity of the lung for carbon monoxide (DL ) and total lung capacity declines, and more frequent CTD development during follow-up periods. The prognosis of the IPAF was better than that of the non-IPAF-IIP and similar to that of the CTD-ILD. IPAF was associated with better prognosis in the IIP cohort on univariate but not on multivariate analysis. Usual interstitial pneumonia (UIP) pattern, old age and low DL independently predicted mortality in the IPAF group.
Compared to the non-IPAF-IIP group, the IPAF group had slower lung function declines and more frequent CTD development during follow-up. Although the prognosis of IPAF group was better than that of non-IPAF-IIP group and similar to that of CTD-ILD group, it showed poor prognosis in patients with old age, UIP pattern, and low DL .
最近将不符合结缔组织病（CTD）标准的具有自身免疫特征的特发性间质性肺炎（IIP）定义为具有自身免疫特征的间质性肺炎（IPAF）。然而，其长期临床过程和结局尚不清楚。
我们纳入了连续诊断为 IIP（n=586）或 CTD 相关间质性肺病（CTD-ILD，n=149）的患者。根据最近的指南，一些 IIP 患者被重新分类为 IPAF。
中位随访时间为 45 个月。在 IIP 患者中，109 例（18.6%）被重新分类为 IPAF。与非 IPAF-IIP 组相比，IPAF 组的一氧化碳弥散量（DL）和总肺容量下降较慢，随访期间更频繁发生 CTD。IPAF 的预后优于非 IPAF-IIP，与 CTD-ILD 相似。在单变量分析中，IPAF 与 IIP 队列的更好预后相关，但在多变量分析中则不然。间质性肺炎（UIP）模式、高龄和低 DL 独立预测 IPAF 组的死亡率。
与非 IPAF-IIP 组相比，IPAF 组在随访期间肺功能下降较慢，更频繁发生 CTD。尽管 IPAF 组的预后优于非 IPAF-IIP 组，与 CTD-ILD 组相似，但在高龄、UIP 模式和低 DL 的患者中预后较差。
