Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College of Huazhong University of Science and Technology, China.
Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College of Huazhong University of Science and Technology, China.
Semin Arthritis Rheum. 2022 Aug;55:152043. doi: 10.1016/j.semarthrit.2022.152043. Epub 2022 Jun 7.
There is a lack of documented real-world evidence about the efficacy of current therapeutics for autoimmune inflammatory rheumatic diseases (AIIRD)-associated adult macrophage activation syndrome (MAS).
To analyze the efficacy of different treatments, especially plasma exchange (PE), in AIIRD-associated MAS.
Among 5775 patients with AIIRD in Tongji Hospital from 2014 to 2020, 62 AIIRD-associated MAS cases were collected. Unadjusted logistic regression, least absolute shrinkage and selection operator (LASSO), and inverse probability of treatment weight (IPTW) analyses were used to characterize the clinical features and potential factors related to the prognosis. Paired t-test was used to compared the changes of inflammatory indicators before and after PE treatment.
The baseline data was defined as the data collected at the onset of MAS, and all of the 62 patients were diagnosed as AIIRD before MAS onset. The prevalance rate of MAS in AIIRD was 1.1%, and the most common types of AIIRD were systemic lupus erythematosus (45.2%) and adult-onset Still's disease (33.9%). All 62 MAS patients received glucocorticoids, 87.1% patients used at least one immunosuppressive agent, and 54.8% received PE. LASSO regression indicates a positive effect of PE on the basis of variables. After PE treatment, serum levels of multiple inflammatory cytokines were rapidly reduced, accompanied by improvements in clinical symptoms and laboratory indecies including ferritin, lactate dehydrogenase, and C-reactive protein. LASSO regression indicates that PE treatment was associated with a marked reduction of mortality (from 53.6% to 11.8%), with a hazard ratio (HR) of 0.148 (p < 0.001) after adjustment for confounding factors using IPTW analysis.
With the background therapy of glucocorticoids and immunosuppressive agents, PE is an effective approach to rapidly clear inflammatory cytokines and reduce mortality of AIIRD-associated MAS.
This study provided real-world information on the efficacy of PE in AIIRD-associated MAS.
目前缺乏有关当前治疗自身免疫性炎症性风湿病（AIIRD）相关成人巨噬细胞活化综合征（MAS）疗效的文献记录的真实世界证据。
分析不同治疗方法，尤其是血浆置换（PE）在 AIIRD 相关 MAS 中的疗效。
收集 2014 年至 2020 年同济医院收治的 5775 例 AIIRD 患者中 62 例 AIIRD 相关 MAS 病例。采用非调整逻辑回归、最小绝对收缩和选择算子（LASSO）以及逆概率治疗权重（IPTW）分析来描述与预后相关的临床特征和潜在因素。采用配对 t 检验比较 PE 治疗前后炎症指标的变化。
基线数据定义为 MAS 发病时收集的数据，所有 62 例患者在 MAS 发病前均被诊断为 AIIRD。AIIRD 中 MAS 的患病率为 1.1%，最常见的 AIIRD 类型为系统性红斑狼疮（45.2%）和成人Still 病（33.9%）。62 例 MAS 患者均接受糖皮质激素治疗，87.1%的患者至少使用一种免疫抑制剂，54.8%的患者接受 PE。LASSO 回归表明，在变量的基础上，PE 具有积极作用。PE 治疗后，多种炎症细胞因子的血清水平迅速降低，同时临床症状和实验室指标（包括铁蛋白、乳酸脱氢酶和 C 反应蛋白）也得到改善。LASSO 回归表明，PE 治疗与死亡率的显著降低相关（从 53.6%降至 11.8%），调整混杂因素后 IPTW 分析的危险比（HR）为 0.148（p<0.001）。
在糖皮质激素和免疫抑制剂的背景治疗下，PE 是一种快速清除炎症细胞因子并降低 AIIRD 相关 MAS 死亡率的有效方法。
本研究提供了关于 PE 在 AIIRD 相关 MAS 中的疗效的真实世界信息。
