成人Still病相关巨噬细胞活化综合征的临床特征、预测因素及预后

Clinical Characteristics, Predictive Factors, and Prognosis of Macrophage Activation Syndrome Associated with Adult-Onset Still’s Disease

摘要: 目的：分析成人Still病(Adult-Onset Still’s Disease, AOSD)相关巨噬细胞活化综合征(Macrophage Activation Syndrome, MAS)的临床特征、预测因素及预后。方法：收集安徽医科大学第二附属医院70例AOSD患者，分为AOSD-MAS组(21例患者)和单纯AOSD组(49例患者)，使用T检验、卡方检验、Mann-Whitney检验、多元Logistic回归分析等统计学方法分析两组患者的临床症状、实验室指标、影像学结果、治疗方案和预后。结果：(1) 两组间性别(P = 0.244)及年龄(P = 0.753)无统计学差异，AOSD-MAS组肝损(90.5% vs 59.2%, P = 0.01)、脾肿大(61.9% vs 22.4%, P = 0.001)、骨穿发现噬血现象(38.1% vs 12.2%, P = 0.031)的发生率明显升高；(2) 甘油三酯水平的升高(OR = 0.984, 95% CI: 0.973~0.996, P = 0.011)和血小板计数的减低(OR = 4.343, 95% CI: 1.111~16.970, P = 0.035)是AOSD患者发生MAS的独立预测因素；(3) 在治疗方案上，单纯AOSD组内不同治疗方式的预后存在显著差异(P < 0.001)，使用激素 + 免疫抑制剂 + 生物制剂的患者比使用其他治疗方式的患者预后更好，AOSD-MAS组内不同治疗方式间比较无显著差异(P = 0.428)，相比单纯AOSD组，AOSD-MAS组生物制剂使用频率(33.3% vs 8.2%)更高，免疫抑制剂更多选用环孢素(85.7% vs 4.1%)，丙种球蛋白使用比例(23.8% vs 0%)高。(4) AOSD-MAS的死亡率显著增加(2.0% vs 23.8%, P = 0.028)。结论：甘油三酯水平升高和血小板计数减低是AOSD发生MAS的独立预测因素，在AOSD患者的治疗中，激素 + 免疫抑制剂 + 生物制剂的联合应用提高了患者的生存率，MAS的发生降低了AOSD患者的死亡率。当AOSD患者出现无法解释的肝损加重或肝脾肿大、骨穿可见噬血现象时应警惕MAS的发生。

Abstract: Objective: To analyze and summarize the clinical characteristics, predictors, and prognosis of macrophage activation syndrome (MAS) associated with adult-onset Still’s disease (AOSD) by Objective: To analyze the clinical characteristics, predictive factors, and prognosis of macrophage activation syndrome (MAS) associated with adult-onset Still’s disease (AOSD). Methods: Seventy patients with AOSD were collected from the Second Affiliated Hospital of Anhui Medical University and divided into an AOSD-MAS group (21 patients) and a simple AOSD group (49 patients). Statistical methods, including T-test, Chi-square test, Mann-Whitney test, and multivariate logistic regression analysis, were employed to analyze the clinical symptoms, laboratory indicators, imaging results, treatment plans, and prognosis of the two groups. Results: (1) No statistically significant differences were observed in gender (P = 0.244) and age (P = 0.753) between the two groups. The AOSD-MAS group exhibited significantly higher incidences of liver damage (90.5% vs. 59.2%, P = 0.01), splenomegaly (61.9% vs. 22.4%, P = 0.001), and hemophagocytosis observed in bone marrow aspiration (38.1% vs. 12.2%, P = 0.031). (2) Elevated triglyceride levels (OR = 0.984, 95% CI: 0.973~0.996, P = 0.011) and decreased platelet counts (OR = 4.343, 95% CI: 1.111~16.970, P = 0.035) were identified as independent predictive factors for the development of MAS in AOSD patients. (3) Regarding treatment plans, significant differences in prognosis were observed among different treatment modalities within the simple AOSD group (P < 0.001), with patients receiving a combination of hormones, immunosuppressants, and biologic agents demonstrating better prognosis compared to those receiving other treatments. No significant differences in prognosis were found among different treatment modalities within the AOSD-MAS group (P = 0.428). Compared to the simple AOSD group, the AOSD-MAS group had a higher frequency of biologic agent use (33.3% vs. 8.2%), more frequently selected cyclosporine as an immunosuppressant (85.7% vs. 4.1%), and a higher proportion of intravenous immunoglobulin use (23.8% vs. 0%). (4) The mortality rate was significantly higher in the AOSD-MAS group (2.0% vs. 23.8%, P = 0.028). Conclusions: Elevated triglyceride levels and decreased platelet counts are independent predictive factors for the development of MAS in AOSD patients. In the treatment of AOSD patients, the combined use of hormones, immunosuppressants, and biologic agents improve patient survival rates. The occurrence of MAS decreases the mortality rate in AOSD patients. Vigilance for MAS should be heightened when AOSD patients exhibit unexplained worsening of liver damage or hepatosplenomegaly, or when hemophagocytosis is observed in bone marrow aspiration.