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

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. Furthermore, AOSD patients accompanied with MAS have a higher frequency of relapse, with no difference between age groups.