Several retrospectivee described the association of interstitial lung disease (ILD) and ANCA-associated vasculitis (AAV). However, the relationship between the ILD and mortality in AAV patients have not been established so far. This study aims to estimate the relevance of AAV-associated-ILD (AAV-ILD) and mortality risk by conducting a systematic review and meta-analysis.A comprehensive systematic review was conducted in accordance with the guidelines of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-analyses). PubMed, Embase.com and the Cochrane Library (Wiley) were searched for original observational studies. Summary estimates were derived with a random-effects model and reported as risk ratio (RR), tested for publication bias and heterogeneity. Ten retrospective cohort studies were included, comprising 526 AAV-ILD patients enrolled from 1974 to 2018. Meta-analysis yielded a pooled RR of 2.90 (95% confidence interval 1.77–4.74) for death among those with AAV-ILD compared to control group. UIP pattern was associated with an even poorer prognosis in comparison to non-UIP pattern (RR 4.36, 95% confidence interval 1.14–16.78). Sensitivity analysis suggested that the meta-RR result was not skewed by a single dominant study. ILD might be associated with a higher mortality risk in AAV patients.

一些回顾性研究描述了间质性肺病 (ILD) 和 ANCA 相关血管炎 (AAV) 的关联。然而，迄今为止，尚未确定 ILD 与 AAV 患者死亡率之间的关系。本研究旨在通过系统评价和荟萃分析来估计 AAV 相关 ILD (AAV-ILD) 与死亡风险的相关性。评论和元分析）。搜索 PubMed、Embase.com 和 Cochrane 图书馆 (Wiley) 以寻找原始观察性研究。使用随机效应模型得出汇总估计值，并报告为风险比 (RR)，测试发表偏倚和异质性。包括十项回顾性队列研究，包括 1974 年至 2018 年招募的 526 名 AAV-ILD 患者。 Meta 分析得出与对照组相比，AAV-ILD 患者死亡的汇总 RR 为 2.90（95% 置信区间 1.77-4.74）。与非 UIP 模式相比，UIP 模式与更差的预后相关（RR 4.36，95% 置信区间 1.14-16.78）。敏感性分析表明，meta-RR 结果不受单一主导研究的影响。ILD 可能与 AAV 患者较高的死亡风险相关。敏感性分析表明，meta-RR 结果不受单一主导研究的影响。ILD 可能与 AAV 患者较高的死亡风险相关。敏感性分析表明，meta-RR 结果不受单一主导研究的影响。ILD 可能与 AAV 患者较高的死亡风险相关。