Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Respir Med. 2018 Apr;137:167-175. doi: 10.1016/j.rmed.2018.02.024. Epub 2018 Mar 1.
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 incidence of acute exacerbations (AEs), is not fully clear. The aim of this study was to elucidate the clinical features and prognostic significance of IPAF.
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 our interstitial lung disease database.
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 those without 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 = 0.092, 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).
A diagnosis of IPAF might predict a favorable prognosis and less risk of AEs in patients with CFIP.
间质性肺疾病具有异质性，慢性纤维性间质性肺炎（CFIP）患者常有临床、血清学和形态学特征提示，但不足以诊断为结缔组织疾病。最近，提出了具有自身免疫特征的间质性肺炎（IPAF）的概念，作为此类患者的平台。然而，IPAF 的预后作用，包括急性加重（AE）的累积发生率，尚不完全清楚。本研究旨在阐明 IPAF 的临床特征和预后意义。
我们在间质性肺病数据库中回顾性探讨了 194 例 CFIP 患者（包括特发性肺纤维化（IPF）患者 163 例和非特异性间质性肺炎（NSIP）患者 31 例）中 IPAF 诊断的临床特征和预后相关性。
16%的 CFIP 患者（IPF 患者的 8%，NSIP 患者的 61%）符合 IPAF 标准。与无 IPAF 患者相比，有 IPAF 的患者明显更年轻，且包括更高比例的女性、从不吸烟者和 NSIP 患者。形态学域是 IPAF 患者最常见的（97%），其次是血清学域（72%）和临床域（53%）。与无 IPAF 的患者相比，有 IPAF 的 CFIP 患者的总生存率（OS；P<0.001，对数秩检验）和 AE 发生率（P=0.029，Gray 检验）更有利。在亚组分析中，有 IPAF 的 NSIP 患者的生存明显优于无 IPAF 的患者（P=0.031，对数秩检验），有 IPAF 的 IPF 患者的 OS 也明显优于无 IPAF 的患者（P=0.092，对数秩检验）。然而，在 IPF 和 NSIP 亚组中，有 IPAF 和无 IPAF 的患者的 AE 发生率无显著差异。此外，符合 IPAF 标准是 OS（风险比（HR）0.127；95%置信区间（CI）0.017-0.952；P=0.045）和 AE 发生率（HR 0.225：95%CI 0.054-0.937；P=0.040）的独立预测因子。
在 CFIP 患者中，IPAF 的诊断可能预示着预后良好和 AE 风险降低。
