Department of Rheumatology, Fukushima Medical University School of Medicine.
Department of Rheumatology, Ohta-Nishinouchi Hospital.
Tohoku J Exp Med. 2021 Nov;255(3):195-202. doi: 10.1620/tjem.255.195.
The aim of this study was to compare the characteristics of Japanese patients with elderly-onset Adult-onset Still's disease (AOSD) and those with younger-onset AOSD. Patients were classified into elderly-onset (≥ 65 years, n = 20) and younger-onset (< 65 years, n = 62) groups according to age at AOSD diagnosis. Analyses included the comparison of clinical features, treatments, and Pouchot and modified Pouchot (mPouchot) scores between the two groups. The frequencies of sore throat, lymphadenopathy, and splenomegaly were significantly lower in the elderly-onset group than in the younger-onset group (30.5% vs. 80.6%, p = 0.0004; 15.0% vs. 54.8%, p = 0.0019; 30.0% vs. 61.3%, p = 0.0203; respectively). There were no significant differences in the frequencies of complications, such as macrophage activation syndrome and disseminated intravenous coagulation, between the patients with elderly-onset or younger-onset AOSD. Serum ferritin levels were higher in the elderly-onset group than in the younger-onset group, albeit without statistical significance (median, 9,423 vs. 4,164 ng/mL, p = 0.1727). Pouchot score was lower in the elderly-onset group than in the younger-onset group (median score, 5.5 vs. 4.0, p = 0.0008); however, there was no significant difference in the mPouchot score between the two groups. Our analyses revealed that elderly-onset AOSD was associated with certain characteristics that were distinct from those of younger-onset AOSD and that the disease severity in patients with elderly-onset AOSD, determined by Pouchot score at the time of AOSD diagnosis, was similar to or less than that in patients with younger-onset AOSD.
本研究旨在比较老年发病型成人Still 病（AOSD）与青年发病型 AOSD 患者的特征。根据 AOSD 诊断时的年龄，将患者分为老年发病型（≥65 岁，n=20）和青年发病型（＜65 岁，n=62）。分析包括比较两组患者的临床特征、治疗方法以及 Pouchot 评分和改良 Pouchot（mPouchot）评分。老年发病型组的咽痛、淋巴结肿大和脾肿大频率显著低于青年发病型组（30.5% vs. 80.6%，p=0.0004；15.0% vs. 54.8%，p=0.0019；30.0% vs. 61.3%，p=0.0203）。老年发病型和青年发病型 AOSD 患者的并发症（如巨噬细胞活化综合征和弥漫性血管内凝血）频率无显著差异。老年发病型组的血清铁蛋白水平高于青年发病型组，但无统计学意义（中位数 9423 与 4164ng/mL，p=0.1727）。老年发病型组的 Pouchot 评分低于青年发病型组（中位数评分 5.5 与 4.0，p=0.0008）；但两组间的 mPouchot 评分无显著差异。本研究结果表明，老年发病型 AOSD 具有与青年发病型 AOSD 不同的特征，老年发病型 AOSD 患者的疾病严重程度（根据 AOSD 诊断时的 Pouchot 评分判断）与青年发病型 AOSD 患者相似或较轻。
