摘要：背景：間質性肺疾病臨床表現多種多樣，慢性纖維化間質性肺炎(CFIP)患者經常具有結締組織病的臨床、血清學和形態學特征但又不足以診斷結締組織病。近來，針對這類患者，有自身免疫特征的間質性肺炎(IPAF)的概念被提出。然而，有自身免疫特征的間質性肺炎的預後，包括急性加重期(AEs)的發生率，尚不完全清楚。本研究的目的在於闡明有自身免疫特征的間質性肺炎的臨床特征和預後意義。方法：回顧性分析194例慢性纖維化間質性肺炎患者中有自身免疫特征的間質性肺炎的臨床特點及預後相關性，其中特發性肺纖維化163例，非特異性間質性肺炎31例。結果：16%的慢性纖維化間質性肺炎患者(其中8%的IPF患者，61%的NSIP患者)符合有自身免疫特征的間質性肺炎標準。與無自身免疫特征的間質性肺炎患者相比,有自身免疫特征的間質性肺炎患者為女性、無吸煙史及符合NSIP者更年輕。有自身免疫特征的間質性肺炎患者影像學差異最大(97%)，其次為血清學差異(72%)和臨床特征差異(53%)。符合有自身免疫特征的間質性肺炎的慢性纖維化間質性肺炎患者在總體生存率方麵預後較好(OS;P<0.001，對數秩檢驗)和副作用發生率較低(P=0.029，格雷檢驗)。在亞組分析中，符合有自身免疫特征的間質性肺炎的NSIP患者的生存率明顯優於無自身免疫特征的間質性肺炎的患者(P=0.031，對數秩檢驗)，符合有自身免疫特征的間質性肺炎的IPF患者的生存率往往優於無有自身免疫特征的間質性肺炎的患者(P=0.0092，對數秩檢驗)。然而，IPF組和NSIP組的不良反應發生率無顯著性差異。此外，達到有自身免疫特征的間質性肺炎標準是生存率[危險比(HR)0.127;95%置信區間(CI)0.017-0.952;P=0.045]和不良反應發生率(HR=0.225;95%CI 0.054-0.937;P=0.040)的獨立預測因素。結論：有自身免疫特征的間質性肺炎的診斷可能預示著慢性纖維化間質性肺炎患者良好的預後和較低的不良事件風險。

        附原文:Backgrounds: Interstitial lung diseases are heterogeneous, and patients with chronic fibrosing interstitial pneumonia (CFIP) often have clinical, serologic, and morphologic features suggestive but not diagnostic of connective tissue disease. Recently, the concept of interstitial pneumonia with autoimmune features (IPAF) has been proposed as a platform for such patients. However, the prognostic role of IPAF, including the cumulative incidece of acute exacerbations (AEs), is not fully clear. The aim of this study was to elucidate the clinical features and prognostic significance of IPAF.Methods: The clinical characteristics and prognostic relevance of a diagnosis of IPAF were retrospectively explored in 194 patients with CFIP, including 163 with idiopathic pulmonary fibrosis (IPF) and 31 with nonspecific interstitial pneumonia (NSIP), in out interstitial lung disease database.Results: Sixteen percent of patients with CFIP (8% of IPF, 61% of NSIP) met the criteria for IPAF. Patients with IPAF were significantly younger and included a higher proportion of women, never-smokers, and patients with NSIP than thosewithout IPAF. The morphologic domain was the most common in patients with IPAF (97%), followed by the serologic domain (72%) and clinical domain (53%). CFIP patients with IPAF had a more favorable prognosis with regard to overall survival (OS; P<0.001, log-rank test) and incidence of AEs (P=0.029, Gray's test) than those without IPAF. In the subgroup analysis, NSIP patients with IPAF had significantly better survival than those without IPAF (P=0.031, log-rank test), and IPF patients with IPAF tended to have better OS than those without IPAF (P=0.0092, log-rank test). However, there were no significant differences in the incidence of AEs between patients with IPAF and those without IPAF in the IPF and NSIP subgroups. Furthermore, fulfilment of the IPAF criteria was an independent predictor of OS [hazard ratio (HR) 0.127; 95% confidence interval (CI) 0.017-0.952; p=0.045) and incidence of AEs (HR 0.225; 95% CI 0.054-0.937; P=0.040).Conclusions: A diagnosis of IPAF might predict a favorable prognosis an less risk of AEs in patients with CFIP.

        引自：Katsuhiro Y, Masato K, Yasunori E, et al. Distinctive characteristics and prognostic significance of interstitial pneumonia with autoimmune features in patients with chronic fibrosing interstitial pneumonia. Respiratory Medicine, 2018,137: 167-175. DOI: 10.1016/j.rmed.2018.02.024.