Department of Rheumatology, Renji Hospital, Shanghai Jiao Tong University, School of Medicine, Shanghai, 200001, China.
Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiao Tong University, School of Medicine, Shanghai, 200001, China.
Clin Rheumatol. 2020 Aug;39(8):2379-2386. doi: 10.1007/s10067-020-04949-0. Epub 2020 Mar 4.
To explore the clinical features, treatments, and prognostic factors of adult-onset Still's disease (AOSD)-associated macrophage activation syndrome (MAS), we conducted a multicenter retrospective clinical study of AOSD-associated MAS patients.
AOSD patients were collected from six tertiary hospitals in China. Medical charts were reviewed and clinical information was recorded and analyzed.
There were 447 AOSD patients enrolled into this retrospective clinical study. Among them, 55 were diagnosed with MAS. Liver dysfunction was the most reliable predictive factor for the screening of MAS in AOSD patients (OR = 75.744, 95%CI = 23.015-249.284, p < 0.0001). In multivariate analysis, clinical features including platelets < 100 × 10/L (OR = 9.546, p = 0.005), aspartate transaminase (AST) > 120 U/L (OR = 25.853, p < 0.0001), triglycerides > 3 mmol/L (OR = 12.9833, p = 0.011)), ferritin > 1500 ng/mL (OR = 5.513, p = 0.050), as well as hemophagocytosis in bone puncture (OR = 18.132, p = 0.001) were highly associated with the occurrence of MAS. The mortality rate of total AOSD patients was 4.47%, MAS was the main cause of death in AOSD patients (OR = 11.705, 95%CI = 4.783-28.647, p < 0.0001). PLT ≤ 100 × 10/L (p = 0.0001), fibrinogen < 1.5 g/L (p = 0.0286), splenomegaly (p = 0.0002), and liver dysfunction (p = 0.0008) highly suggested poor prognosis.
MAS occurrence is the major cause of death in AOSD patients. Notable liver dysfunction, as well as splenomegaly, low number of platelets or neutrophils, high levels of serum ferritin, and reduced level of fibrinogen are risk factors for poor outcome. Key Points • This is a multicenter retrospective study of AOSD-associated MAS with large number of cases.
为了探讨成人斯蒂尔病（AOSD）相关巨噬细胞活化综合征（MAS）的临床特征、治疗方法和预后因素，我们对 AOSD 相关 MAS 患者进行了一项多中心回顾性临床研究。
我们从中国六家三级医院收集了 AOSD 患者。对病历进行了回顾，并记录和分析了临床资料。
本回顾性临床研究共纳入 447 例 AOSD 患者，其中 55 例被诊断为 MAS。肝功能障碍是 AOSD 患者 MAS 筛查最可靠的预测因素（OR=75.744，95%CI=23.015-249.284，p<0.0001）。多因素分析显示，临床特征包括血小板计数<100×10/L（OR=9.546，p=0.005）、天冬氨酸转氨酶（AST）>120 U/L（OR=25.853，p<0.0001）、甘油三酯>3 mmol/L（OR=12.9833，p=0.011）、铁蛋白>1500 ng/mL（OR=5.513，p=0.050）以及骨髓穿刺中存在噬血细胞现象（OR=18.132，p=0.001）与 MAS 的发生高度相关。AOSD 患者的总死亡率为 4.47%，MAS 是 AOSD 患者死亡的主要原因（OR=11.705，95%CI=4.783-28.647，p<0.0001）。血小板计数≤100×10/L（p=0.0001）、纤维蛋白原<1.5 g/L（p=0.0286）、脾肿大（p=0.0002）和肝功能障碍（p=0.0008）高度提示预后不良。
MAS 的发生是 AOSD 患者死亡的主要原因。显著的肝功能障碍，以及脾肿大、血小板或中性粒细胞计数低、血清铁蛋白水平高、纤维蛋白原水平降低是不良预后的危险因素。
这是一项多中心、回顾性的 AOSD 相关 MAS 研究，病例数较多。
