Article Title

Treatment outcomes in a 10-year geographic cohort of patients with Adult-onset Still’s Disease: Case series and literature review

Abstract

Introduction: Adult-onset Stills Disease (AOSD) is a rare immuno-inflammatory disease with an unpredictable course. Contemporary treatment with biological agents is commonplace but the efficacy of treatment and effects on long-term outcomes are poorly defined. We investigated prognostic markers and evaluated outcomes in an Australian cohort of hospitalized patients with AOSD diagnosed and treated over 10 years.

Methods: Patients greater than 18 years of age with a discharge ICD-10 code for AOSD were selected from medical records at 3 tertiary hospitals in Perth, Western Australia from 2009-2019. Demographic data, and clinical and serological outcomes after 12 months of follow-up were collected. Relationships between plasma ferritin and clinical remission, flare rate, need for conventional treatment with or without conventional or biological disease-modifying agents (csDMARD and bDMARD) and Physician Global Assessment score (PGA) were evaluated, as were the effect of treatments on serologic markers of remission and PGA. Fishers exact tests for a categorical comparisons and Mann-Whitney U-tests for comparison of continuous outcomes were performed. P < 0.05 was considered statistically significant.

Results: All patients received corticosteroid therapy and 18 of 24 also received csDMARDs. Of these, 8 progressed to bDMARDs. Lower baseline ferritin concentrations were observed in those who achieved complete remission, required csDMARDs +/- bDMARDs, or had more flares per 100 patient-years and lower PGA scores, but the results were not statistically significant. Serological markers of remission decreased markedly with all treatments, but no significant differences between treatments or in patients achieving remission was observed.

Conclusions: Long term follow-up was limited but remission rates appeared low. No unequivocal predictors of treatment response or outcome were identified. A trend towards greater use of csDMARDs and bDMARDs, and low PGA scores (poor outcome at 12 months), implying worse outcomes was observed with hyperferritinemia. Accordingly, marked hyperferritinemia may be a biomarker for more sustained or severe AOSD manifestations and predispose to more relapses. Further research is required to examine this possibility in more detail.

Keywords

case series, literature review, Adult-onset Still’s Disease, treatment outcomes

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