Background/Purpose: There is no randomized controlled trial data to guide us for the management of Adult Still’s Disease (AOSD) patients refractory to conventional treatments. We herein aimed to analyze retrospectively the treatment results of a series of refractory AOSD patients, who received different biologics.

Methods: We screened our database for patients diagnosed with AOSD between 1987-2015,and reviewed charts of those patients refractory to corticosteroid and DMARD combination and received biologics including TNF, IL-1 and IL-6 inhibitors. Patients were grouped according to their first biologics as TNF-inhibitors (TNF-i) or others (anakinra (ANK) or tocilizumab (TCZ)).We also analyzed the patient responses according to their disease characteristics as polycyclic or chronic patients. Time to remission were analysed by Kaplan-Meier method, and the association of response with first biological treatment was analysed by regression analysis.

Results: Twenty-four patients with refractory AOSD (chronic:9 and polycyclic:15),who received biologic with a mean duration of 41±27 months. The first biological treatment was TNF-i in 14 and other agents in 10 (ANK in 7 and TCZ in 3).Of the patients,50% were switched from TNF-i to ANK or TCZ. Comparison of the patients according to their first biologic therapy is given in Table 1.The patients who received treatment with other biologics were associated with a better response as the first treatment and tended to achieve remission earlier compared with those who received TNF-i as their first biologicals (median time to remission 4 vs.8 months, log-rank p=0.44).

Joint involvement was more common and skin involvement tended to be more common in the chronic course group (100% vs 50%,78% vs 36%, respectively). While the duration of biologic treatment tended to be longer in the chronic course group (51mo vs 28mo,p=0.04), duration of remission was similar between the groups (26mo vs 20mo, p=0.4). The remission rate tended to be higher in the polycyclic group, whereas CRP and ESH tended to be lower in the chronic group.

Complete remission rates under biologics course were as follows: 70% with TCZ 8mg/kg(n=10), 56% with ANK 100-200mg/d(n=16), 25% with etanercept (n=12), and 18% with infliximab (n=11) treatments. Regression analysis showed that selection of TCZ or ANK biologics as the first biologic was associated with a better response independent of the disease course type(estimate 2.5, 95% CI 0.3-4.7, p=0.026).

No serious infection was observed in patients treated with biologics. In two patients severe skin reactions occurred with ANK and infliximab and, in one patient an immune thrombocytopenia developed under TCZ treatment.

Table 1.Comparison of study variables according to the first biologic agents

Conclusion: This case series of AOSD patients suggest that ANK and TCZ seem to be better treatment choices in terms of remission rate and time to remission in patients refractory to conventional treatments.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/biologic-agents-in-refractory-adult-stills-disease-better-response-rates-and-acceptable-safety-with-anakinra-and-tocilizumab/