摘要：背景：间质性肺疾病临床表现多种多样，慢性纤维化间质性肺炎(CFIP)患者经常具有结缔组织病的临床、血清学和形态学特征但又不足以诊断结缔组织病。近来，针对这类患者，有自身免疫特征的间质性肺炎(IPAF)的概念被提出。然而，有自身免疫特征的间质性肺炎的预后，包括急性加重期(AEs)的发生率，尚不完全清楚。本研究的目的在于阐明有自身免疫特征的间质性肺炎的临床特征和预后意义。方法：回顾性分析194例慢性纤维化间质性肺炎患者中有自身免疫特征的间质性肺炎的临床特点及预后相关性，其中特发性肺纤维化163例，非特异性间质性肺炎31例。结果：16%的慢性纤维化间质性肺炎患者(其中8%的IPF患者，61%的NSIP患者)符合有自身免疫特征的间质性肺炎标准。与无自身免疫特征的间质性肺炎患者相比,有自身免疫特征的间质性肺炎患者为女性、无吸烟史及符合NSIP者更年轻。有自身免疫特征的间质性肺炎患者影像学差异最大(97%)，其次为血清学差异(72%)和临床特征差异(53%)。符合有自身免疫特征的间质性肺炎的慢性纤维化间质性肺炎患者在总体生存率方面预后较好(OS;P<0.001，对数秩检验)和副作用发生率较低(P=0.029，格雷检验)。在亚组分析中，符合有自身免疫特征的间质性肺炎的NSIP患者的生存率明显优于无自身免疫特征的间质性肺炎的患者(P=0.031，对数秩检验)，符合有自身免疫特征的间质性肺炎的IPF患者的生存率往往优于无有自身免疫特征的间质性肺炎的患者(P=0.0092，对数秩检验)。然而，IPF组和NSIP组的不良反应发生率无显著性差异。此外，达到有自身免疫特征的间质性肺炎标准是生存率[危险比(HR)0.127;95%置信区间(CI)0.017-0.952;P=0.045]和不良反应发生率(HR=0.225;95%CI 0.054-0.937;P=0.040)的独立预测因素。结论：有自身免疫特征的间质性肺炎的诊断可能预示着慢性纤维化间质性肺炎患者良好的预后和较低的不良事件风险。

        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.