Abstract
BackgroundAdult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disease which encompasses patients with heterogenous presentation and a wide range of clinical courses. In this study, we aimed to identify potential subgroups of AOSD and reveal risk factors for relapse.MethodsWe included a total of 216 AOSD patients who received treatment in nine hospitals between 2000 and 2019. All patients fulfilled the Yamaguchi classification criteria. We retrospectively collected information about baseline characteristics, laboratory tests, treatment, relapse, and death. We performed latent class analysis and time-to-event analysis for relapse using the Cox proportional hazard model.ResultsThe median age at disease onset was 51.6 years. The median follow-up period was 36.8 months. At disease onset, 22.3% of the patients had macrophage activation syndrome. The median white blood cell count was 12,600/μL, and the median serum ferritin level was 7230 ng/mL. Systemic corticosteroids were administered in all but three patients (98.6%) and the median initial dosage of prednisolone was 40mg/day. Ninety-six patients (44.4%) were treated with concomitant immunosuppressants, and 22 (10.2%) were treated with biologics. Latent class analysis revealed that AOSD patients were divided into two subgroups: the typical group (Class 1: 71.8%) and the elderly-onset group (Class 2: 28.2%). During the follow-up period, 13 of 216 patients (6.0%) died (12 infections and one senility), and 76 of 216 patients (35.1%) experienced relapses. Overall and relapse-free survival rates at 5 years were 94.9% and 57.3%, respectively, and those rates were not significantly different between Class 1 and 2 (p=0.30 and p=0.19). Time-to-event analysis suggested higher neutrophil count, lower hemoglobin, and age ≥65 years at disease onset as risk factors for death and age ≥65 years at disease onset as a risk factor for relapse.ConclusionsAOSD patients were divided into two subgroups: the typical group and the elderly-onset group. Although the survival of patients with AOSD was generally good, the patients often experienced relapses. Age ≥65 years at disease onset was the risk factor for relapse.
Highlights
Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disease which encompasses patients with heterogenous presentation and a wide range of clinical courses
A five-fold increase of serum ferritin levels compared with the normal value is strongly suggestive of AOSD, and ferritin is considered a useful marker of disease activity of AOSD [11, 12]
We collected the results of blood examinations at diagnosis, which included white blood cell (WBC) count, neutrophil count, hemoglobin (Hb), platelet count, erythrocyte sedimentation rate (ESR), aspartate aminotransferase (AST), alanine aminotransferase (ALT), lactate dehydrogenase (LDH), C-reactive protein (CRP), and ferritin
Summary
Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disease which encompasses patients with heterogenous presentation and a wide range of clinical courses. In 1897, George Frederic Still first reported juvenile chronic arthritis with fever and rash as Still’s disease [4], which is considered systemic juvenile idiopathic arthritis (sJIA). Adult Still’s disease (ASD) was reported by Eric Bywaters as a series of adult patients who had features similar to the children with sJIA with three main symptoms: quotidian fevers, arthritis, and evanescent rash and did not fulfill the criteria for classic rheumatoid arthritis [5]. AOSD patients do not always present a five-fold increase of serum ferritin levels and other manifestations of AOSD are non-specific. Considering the lack of specific disease markers, patients diagnosed as AOSD could be a heterogeneous population
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