Background 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 summarise 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 (PFT). Results Ten articles were included, comprising 514 patients: 67.8% female, mean age 52.4years (SD4.6). Baseline high-resolution computed tomography 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 estimates, after meta-analysis, for baseline Forced Vital Capacity (FVC) was 60.8% predicted (SE 2.1), and Diffusion Capacity of Lungs for Carbon Monoxide (DLco) was 49.8% (SE 3.5). After one-year, pooled improvement in FVC was 14.1% from baseline (SE 3.1) and in DLco was 15.1% (SE 2.8). Paired t-test demonstrated significant overall improvement in FVC (p = 0.007) and DLco (p = 0.002). Patients receiving RTX had 12.2% improvement in FVC and 2.9% increase in DLco at one-year; for patients receiving CYC, there was 17% improvement and 6.3% increase, respectively. 28 deaths were reported. Conclusion Our SLR, the first to summarise 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疾病的治疗和结果：系统文献综述


背景抗合成酶综合征（ASS）是一种慢性自身免疫性疾病，其中间质性肺疾病（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）的治疗和结果。方法 在数据库中搜索 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审员进行。在适当的情况下，使用随机效应模型和配对 t 检验进行荟萃分析，以评估肺功能测试 (PFT) 的治疗后变化。结果纳入 10 篇文章，包括 514 名患者：67.8% 女性，平均年龄 52.4 岁（SD4.6）。 447 名患者 (86.9%) 记录了基线高分辨率计算机断层扫描；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。经过荟萃分析后，基线用力肺活量 (FVC) 的汇总估计值为预测值的 60.8% (SE 2.1)，肺一氧化碳扩散能力 (DLco) 为 49.8% (SE 3.5)。一年后，FVC 与基线相比的综合改善为 14.1% (SE 3.1)，DLco 为 15.1% (SE 2.8)。配对 t 检验显示 FVC (p = 0.007) 和 DLco (p = 0.002) 整体显着改善。接受 RTX 治疗的患者一年时 FVC 改善 12.2%，DLco 增加 2.9%；接受 CYC 治疗的患者，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 首次总结了 ASS-ILD 的治疗和结果，未发现治疗效果之间存在决定性差异。 需要更强有力的试验来降低 ASS-ILD 的发病率和死亡率。