Clinical characteristics and outcome of elderly onset adult-onset Still's disease: A 10-year retrospective study

IF 4.7 Q2 IMMUNOLOGY Journal of Translational Autoimmunity Pub Date : 2023-01-01 DOI:10.1016/j.jtauto.2023.100196
Sheng Li , Shuni Ying , Juan Bai , Yuqian Wang, Changyi Yang, Qingmiao Sun, Hong Fang, Jianjun Qiao
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引用次数: 1

Abstract

Objective

Our objective was to retrospectively analyze the clinical characteristics and outcome of adult-onset Still's disease (AOSD) patients with elderly onset.

Methods

Retrospective data of patients diagnosed with AOSD in our institute during 2013–2021 were analyzed. The diagnoses were based on the Yamaguchi criteria for AOSD. All long-term follow-up data were collected from medical records and phone calls.

Results

In total, 281 AOSD patients were enrolled in this study, with the median follow-up interval of 47 months. Thirty-two (11.4%, ≥65 years) AOSD patients were classified into the elderly onset groups. Compared to the younger onset group, the percentage of patients with skin rash (p = 0.047), sore throat (p = 0.001), myalgia (p = 0.001), splenomegaly (p = 0.039), hepatosplenomegaly (p = 0.002) and the Pouchot's score (p = 0.002) were significantly lower in the elderly onset group. The death rate (p = 0.014) of elderly onset group is higher than younger onset group, and the independent risk factors of mortality in all AOSD patients were age at onset (HR: 1.115, p = 0.044), disseminated intravascular coagulation (HR: 391.576, p = 0.001) and pleuritis (HR: 23.162, p = 0.033). The probability of relapse was significantly increased in the patients with macrophage activation syndrome (MAS) compared with the patients without MAS (p < 0.001), though the different age groups of AOSD patients with MAS showed no difference in the probability of relapse (p = 0.737).

Conclusion

Elderly onset AOSD patients were distinguished by several distinct clinical features compared to younger onset AOSD patients. The frequency of relapse and complications were similar to that of AOSD patients with elderly or younger onset. A higher mortality rate was observed in elderly onset AOSD patients, and the mortality of AOSD patients was related to age at onset, DIC and pleuritis.

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老年发病成人发病斯蒂尔氏病的临床特点和预后:一项10年回顾性研究
目的回顾性分析老年起病成人起病斯蒂尔氏病(AOSD)患者的临床特点和转归。方法回顾性分析我院2013-2021年诊断为AOSD的患者资料。诊断依据AOSD的Yamaguchi标准。所有的长期随访数据都是从医疗记录和电话中收集的。结果共纳入281例AOSD患者,中位随访时间为47个月。32例(11.4%,≥65岁)AOSD患者分为老年起病组。与年轻起病组相比,老年起病组出现皮疹(p = 0.047)、喉咙痛(p = 0.001)、肌痛(p = 0.001)、脾肿大(p = 0.039)、肝脾肿大(p = 0.002)和Pouchot评分(p = 0.002)的比例显著降低。老年发病组的死亡率(p = 0.014)高于年轻发病组,且所有AOSD患者死亡的独立危险因素为发病年龄(HR: 1.115, p = 0.044)、弥散性血管内凝血(HR: 391.576, p = 0.001)和胸膜炎(HR: 23.162, p = 0.033)。巨噬细胞活化综合征(MAS)患者的复发概率明显高于无MAS患者(p <0.001),但不同年龄组的AOSD合并MAS患者的复发概率没有差异(p = 0.737)。结论老年AOSD与年轻AOSD有明显的临床特征。复发及并发症发生率与老年或低龄AOSD患者相似。高龄AOSD患者病死率较高,且病死率与发病年龄、DIC、胸膜炎有关。
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来源期刊
Journal of Translational Autoimmunity
Journal of Translational Autoimmunity Medicine-Immunology and Allergy
CiteScore
7.80
自引率
2.60%
发文量
33
审稿时长
55 days
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