School of Population and Global Health, University of Western Australia, Crawley, Western Australia, Australia
Department of Intensive Care Medicine, Northern Hospital, Epping, Victoria, Australia.
BMJ Open. 2019 Dec 11;9(12):e031444. doi: 10.1136/bmjopen-2019-031444.
To clarify the prognosis and prognostic factors of interstitial pneumonia with autoimmune features (IPAF) in comparison to idiopathic pulmonary fibrosis (IPF), the most common idiopathic interstitial pneumonia, and connective tissue disease-associated interstitial pneumonia (CTD-IP).
A systematic review and meta-analysis.
Electronic databases such as Medline and Embase were searched from 2015 through 6 September 2019.
Primary studies that comparatively investigated the prognosis or prognostic factors of IPAF were eligible.
Two reviewers extracted relevant data and assessed the risk of bias independently. A meta-analysis was conducted using a random-effects model. The quality of presented evidence was assessed by the Grades of Recommendation, Assessment, Development, and Evaluation system.
Out of a total of 656 records retrieved, 12 studies were reviewed. The clinical features of IPAF were diverse between studies, which included a radiological and/or pathological usual interstitial pneumonia (UIP) pattern of between 0% and 73.8%. All studies contained some risk of bias. There was no significant difference of all-cause mortality between IPAF-UIP and IPF in all studies, although the prognosis of IPAF in contrast to IPF or CTD-IP varied between studies depending on the proportion of UIP pattern. Among the potential prognostic factors identified, age was significantly associated with all-cause mortality of IPAF by a pooled analysis of univariate results with a hazard ratio (HR) of 1.06 (95% confidence interval (CI) 1.04 to 1.07). The adjusted effect of age was also significant in all studies. The quality of presented evidence was deemed as very low.
There was no significant difference of all-cause mortality between IPAF-UIP and IPF. Age was deemed as a prognostic factor for all-cause mortality of IPAF. The findings should be interpreted cautiously due to the low quality of the presented evidence.
CRD42018115870.
与最常见的特发性间质性肺炎（特发性肺纤维化，IPF）和结缔组织病相关间质性肺炎（CTD-IP）相比，明确具有自身免疫特征的间质性肺炎（IPAF）的预后及其预后因素。
系统评价和荟萃分析。
从 2015 年至 2019 年 9 月 6 日，检索了 Medline 和 Embase 等电子数据库。
合格的研究为比较 IPAF 的预后或预后因素的原始研究。
两名评审员独立提取相关数据并评估偏倚风险。使用随机效应模型进行荟萃分析。通过推荐、评估、制定和评估系统（Grades of Recommendation, Assessment, Development, and Evaluation system）评估提出证据的质量。
从总共 656 条记录中检索出 12 项研究。由于研究之间的 IPAF 临床特征存在差异，因此包括放射学和/或病理学通常的间质性肺炎（UIP）模式的比例在 0%至 73.8%之间。所有研究都存在一定的偏倚风险。尽管 IPAF 的预后因 UIP 模式的比例而异，但在所有研究中，IPA-FUIP 与 IPF 之间的全因死亡率均无显着差异。在确定的潜在预后因素中，年龄通过对单变量结果的汇总分析与 IPAF 的全因死亡率显著相关，风险比（HR）为 1.06（95%置信区间（CI）为 1.04 至 1.07）。所有研究均表明年龄的调整作用也具有统计学意义。提出证据的质量被认为非常低。
IPA-FUIP 与 IPF 之间的全因死亡率无显着差异。年龄被认为是 IPAF 全因死亡率的预后因素。由于提出证据的质量较低，因此应谨慎解释这些发现。
PROSPERO 注册号：CRD42018115870。
