Objectives Anti-synthetase syndrome (ASS) is a chronic autoimmune condition, with interstitial lung disease (ILD) being a key feature. This systematic literature review (SLR; CRD42023416414) aimed to summarize treatments and outcomes of ILD associated with ASS (ASS-ILD). Methods Databases were searched for articles discussing ASS-ILD management and outcomes, published 1946–September 2023. Screening and data extraction were performed by two reviewers. Meta-analysis, using a random effects model, and paired t-tests were undertaken where appropriate to evaluate post-treatment-change in pulmonary function tests. Results Ten articles were included, comprising 514 patients: 67.8% female, mean age 52.4 years (s.d. 4.6). Baseline high-resolution CT was documented in 447 patients (86.9%); the most common pattern was non-specific interstitial pneumonia (n = 220; 49.2%). The most common myositis-associated autoantibody was anti-Jo1 (48%), with 27.8% having associated anti-Ro52 antibodies. Pooled estimate, after meta-analysis, for baseline forced vital capacity (FVC) was 60.8% predicted (s.e. 2.1) and for diffusion capacity of lungs for carbon monoxide (DLco) was 49.8% (s.e. 3.5). After 1 year, pooled improvement in FVC was 14.1% from baseline (s.e. 3.1) and in DLco was 15.1% (s.e. 2.8). Paired t-test demonstrated significant overall improvement in FVC (P = 0.007) and DLco (P = 0.002). Patients receiving rituximab had 12.2% improvement in FVC and 2.9% increase in DLco at 1 year; for patients receiving CYC, there was 17% improvement and 6.3% increase, respectively. Twenty-eight deaths were reported. Conclusion Our SLR, the first to summarize management and outcomes of ASS-ILD, found no conclusive difference between effectiveness of treatments. More robust trials are required to reduce morbidity and mortality resulting from ASS-ILD.

中文翻译：

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抗合成酶综合征相关间质性肺病的治疗和结局：系统文献综述


目的 抗合成酶综合征 （ASS） 是一种慢性自身免疫性疾病，其中间质性肺病 （ILD） 是一个关键特征。本系统文献综述 （SLR;CRD42023416414） 旨在总结与 ASS 相关的 ILD （ASS-ILD） 的治疗和结局。方法 检索数据库以查找 1946 年至 2023 年 9 月发表的讨论 ASS-ILD 管理和结果的文章。筛选和数据提取由两名评价员进行。在适当的情况下进行 meta 分析、使用随机效应模型和配对 t 检验，以评估肺功能检查的治疗后变化。结果 共纳入 10 篇文章，涉及 514 例患者： 67.8% 为女性，平均年龄 52.4 岁 （s.d. 4.6）。447 例患者 （86.9%） 记录了基线高分辨率 CT;最常见的模式是非特异性间质性肺炎 （n = 220;49.2%）。最常见的肌炎相关自身抗体是抗 Jo1 （48%），其中 27.8% 具有相关的抗 Ro52 抗体。经过荟萃分析，基线用力肺活量 （FVC） 的合并估计值为 60.8% 预测值 （s.e. 2.1），肺一氧化碳弥散量 （DLco） 为 49.8% （s.e. 3.5）。1 年后，FVC 的总体改善比基线 （s.e. 3.1） 高 14.1%，DLco 的总体改善为 15.1% （s.e. 2.8）。配对 t 检验显示 FVC （P = 0.007） 和 DLco （P = 0.002） 的总体改善有统计学意义。接受利妥昔单抗治疗的患者在 1 年时 FVC 改善 12.2%，DLco 增加 2.9%;对于接受 CYC 的患者，分别有 17% 的改善和 6.3% 的增加。据报道，有 28 人死亡。结论我们的 SLR 是第一个总结 ASS-ILD 管理和结局的 SLR，发现治疗效果之间没有决定性的差异。 需要更稳健的试验来降低 ASS-ILD 导致的发病率和死亡率。