Department of Rheumatology and Immunology, People's Hospital of Guangxi Zhuang Autonomous Region, Nanning, China.
Ann Med. 2024 Dec;56(1):2411605. doi: 10.1080/07853890.2024.2411605. Epub 2024 Oct 9.
Rapidly progressive interstitial lung disease (RP-ILD) is a frequent and serious manifestation of idiopathic inflammatory myopathy (IIM) associated with poor outcomes. Plasma exchange (PE) can quickly remove pathogenic substances from the blood. Therefore, PE may be efficacious in IIM patients who have elevated levels of autoantibodies, cytokines and chemokines, fighting for time for immunosuppressive therapy. However, the value of adding PE to immunosuppressants remains unclear. The purpose of this study was to determine the short-term outcomes, including the survival rate at 6 months and change of the laboratory data, of PE in combination with immunosuppressants and/or biologics in the treatment of IIM-RP-ILD.
We searched PubMed, Embase and Cochrane Library to find reports of interest published from inception to March 4, 2024. STATA 15.1 was used for data analysis. A fixed or random-effects model with inverse-variance weighting was used to estimate the pooled risk ratio (RR) and 95% confidence interval (CI).
Two hundred and thirty studies were identified. Eleven studies, including five retrospective cohort studies, four case-control studies and two case series, were included. PE was performed on 114 patients. The survival rate at 6 months was 80% (95%CI = 64%-92%), with moderate heterogeneity (=63.45%, < 0.05). Moreover, the 6-month survival rate was significantly better in the PE group than in the non-PE group (RR, 1.34; 95% CI = 1.05-1.71, =30.7%; = 0.194). ILD-related serum markers, including ferritin, KL-6 and anti-MDA-5 antibody titres, were significantly suppressed by a series of PE treatments ( < 0.05).
The application of PE therapy plus treatment with corticosteroids, immunosuppressants and/or biologics was effective for patients with IIM-RP-ILD. PE may have additional supportive effect in intractable disease.
快速进展性间质性肺病（RP-ILD）是特发性炎性肌病（IIM）的一种常见且严重的表现，与预后不良有关。血浆置换（PE）可迅速从血液中清除致病物质。因此，对于自身抗体、细胞因子和趋化因子水平升高的 IIM 患者，PE 可能有效，为免疫抑制治疗争取时间。然而，在免疫抑制剂中添加 PE 的价值仍不清楚。本研究的目的是确定 PE 联合免疫抑制剂和/或生物制剂治疗 IIM-RP-ILD 的短期结果，包括 6 个月时的生存率和实验室数据的变化。
我们检索了 PubMed、Embase 和 Cochrane Library，以查找截至 2024 年 3 月 4 日发表的相关报告。使用 STATA 15.1 进行数据分析。采用固定或随机效应模型进行逆方差加权，以估计合并风险比（RR）和 95%置信区间（CI）。
共确定了 230 项研究。纳入了 11 项研究，包括 5 项回顾性队列研究、4 项病例对照研究和 2 项病例系列研究。共对 114 例患者进行了 PE。6 个月时的生存率为 80%（95%CI=64%-92%），存在中度异质性（=63.45%， < 0.05）。此外，PE 组的 6 个月生存率明显优于非 PE 组（RR，1.34；95%CI=1.05-1.71， =30.7%； = 0.194）。一系列 PE 治疗显著抑制了ILD 相关血清标志物，包括铁蛋白、KL-6 和抗 MDA-5 抗体滴度（ < 0.05）。
PE 治疗联合皮质类固醇、免疫抑制剂和/或生物制剂的应用对 IIM-RP-ILD 患者有效。PE 可能对难治性疾病具有额外的支持作用。
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