Background: Even though corticosteroids and cDMARDs are effective for inducing remission in patients with Adult-Onset Still Disease (AOSD), relapse is common. Hence, maintaining the clinical stability is challenging. Almost all of the patients face side effects because of high dose steroid treatment. Biological DMARDs have been reported to be effective in refractory patients.

Objectives: We aimed to evaluate the patients’ outcomes who were diagnosed with AOSD and treated with at least one bDMARDs in our tertiary center.

Methods: Patients with AOSD who were followed in our clinic between 2007 and 2020 were screened retrospectively. For the diagnosis of AOSD, all of the patients fulfilled Yamaguchi criteria. The demographic characteristics, baseline and post-treatment clinical findings and outcomes were reported.

Results: Twenty-eight patients (21 F, 7 M) were screened ( Figure 1 ). The mean disease duration of the first bDMARD was 21,76 ± 28,05 months (mean ± SD). The mean duration of bDMARD treatment was 37,04 ± 30,75 months. The reasons for starting a bDMARD were systemic symptoms (%80) and chronic arthritis (%20). All of the patients used methotrexate (MTX) except one. This patient had macrophage activation syndrome during diagnosis and was treated with cyclosporine. All of the patients were treated with corticosteroids (34,28 ± 26,70 mg/d) and a cDMARD at initiation (22 of them MTX, 1 of them azathioprine, 2 of them cyclosporine and 1 of them IVIg). Anakinra was the most preferred biologic as a first-line treatment modality (TNF inhibitors=5, tocilizumab=4). The main reason for switching was loss of efficacy (8/22). Tocilizumab was the most used agent in 2 ^nd line and canakinumab was in 3 ^rd line. Twenty-two patients were in remission at last visit. Also, 15 patients were steroid-free, 14 patients were MTX-free. Patient global visual analogue scale, acute phase reactants and daily steroid dose were reduced significantly at last visit compared to the initial visit ( Table 1 ).

Figure 1.

Presenting signs and the symptoms of the patients

bDMARD treatment was terminated in 5 patients due to complete remission (n=2) and side effects (1 of them pneumonia, 1 of them EBER (+) Hodgkin Lymphoma and 1 of them tuberculosis). Six patients experienced local injection site reaction, none of them stopped treatment. Also, one patient died while she was in remission under anakinra treatment with an unknown cause.

Conclusion: The most common presenting symptoms in our cohort were fever and salmon-colored rash. Tocilizumab is an alternative treatment modality in cases with chronic arthritis and IL-1 inhibitors are an alternative for systemic course of disease. bDMARDs, especially IL-1 inhibitors are highly effective for refractory patients with AOSD.

Disclosure of Interests: None declared