Background/Purpose: No data comparing the long-term outcome of Adult-Onset Still’s Disease (AOSD) patients treated with different biological drugs is currently available. We evaluated the efficacy and safety of different biological agents in a cohort of 19 AOSD patients followed-up at a single referral center, and compared the results with the current knowledge. 

Methods: Nineteen Italian AOSD patients were treated with biological agents and followed-up at our institution for at least 24 months between 1998 and 2013. For each case we retrospectively evaluated disease course, efficacy of treatment, and potential adverse effects. Efficacy was evaluated as ‘Complete response’ (CR: absence of articular and systemic manifestations, normalization of inflammatory indexes, >50% reduction in the corticosteroid dosage), ‘Partial response’ (PR: clinical improvement without normalization of inflammatory markers, nor >50% reduction in the dose of steroids), or ‘Treatment failure’ (TF: persistence/worsening of disease manifestations, persistent elevation of inflammatory markers, or need for an increased dose of corticosteroids despite 2 months of treatment). We compared our data with the current literature on the topic. 

Results: Nineteen patients with AOSD refractory to conventional therapies were administered biological agents. The average duration of follow-up after initiation of biologic agents was 5 years. Overall, the different biologic drug regimens induced an improvement of systemic and articular manifestations in 17 (89%) patients. Anakinra was used in all 19 patients; etanercept, tocilizumab and adalimumab were used in 6, 4, and 1 patient, respectively. Fifteen patients responded to anakinra (79%; CR 68%; PR 11%). A minority of patients (4 out of 19, 21%) did not respond to anakinra; three of them (16%) responded to tocilizumab, and one (5%) responded to adalimumab. Etanercept was used unsuccessfully in six patients. A decrease in the dose of corticosteroids and immunosuppressants was possible in all patients (discontinuation: 56%; >25% reduction: 44%). A reduction in the dose of the immunosuppressive drugs was possible in 14 patients (74% of the cohort; discontinuation, 21%; reduction 53%). Three patients experienced herpes zoster reactivation while on treatment. 

Conclusion: Biological agents represent a pivotal therapeutic resource for AOSD patients refractory to conventional treatment and display a good safety profile. IL-1 blockade with anakinra represented the mainstay of treatment. Both anakinra and tocilizumab treatments were more effective than TNF-α blockers. IL-6 blockade may be more effective than IL-1 blockade in a group of patients with chronic articular involvement. Most of the patients in whom treatment with biologics proved poorly effective had already developed irreversible structural damages before the biologic treatment was started. It is thus conceivable that a more prompt initiation of biological agents could be beneficial in patients with severe AOSD.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/efficacy-and-safety-of-biologic-agents-in-adult-onset-stills-disease-a-long-term-follow-up-of-19-patients-at-a-single-referral-center/