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Treatment of refractory adult onset Still’s disease with tocilizumab—a single centre experience and literature review

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Adult-onset Still's disease (AOSD) is defined as a systemic inflammatory disorder of unknown aetiology and is classified as a multigene autoinflammatory disease. Treatment of AOSD still remains mostly empirical with nonsteroidal anti-inflammatory drugs, glucocorticoids and conventional synthetic disease-modifying antirheumatic drugs or cyclosporin A. Inhibitors of tumour necrosis factor-alpha and interleukin-1 (IL-1) antagonists have shown efficacy in certain subsets of patients with AOSD. The IL-6 molecule is one of the potential targets in treating AOSD considering that its level is increased in both the systemic and chronic articular forms of the disease.

We present a series of eight patients from our centre with refractory AOSD treated with tocilizumab (TCZ). The drug was administered intravenously (6–8 mg/kg every 3–4 weeks) or subcutaneously (162 mg weekly). One patient had a disease relapse during TCZ therapy, and the drug had to be withdrawn in one patient due to a severe infection, while five out of six patients currently treated are in stable remission.

Many previous reports have suggested that TCZ is an efficacious option for the treatment of refractory AOSD and the cases presented herein support this finding. A literature search revealed two previous reports of subcutaneous TCZ administration TCZ in AOSD, and our experience supports subcutaneous TCZ as a promising option for treatment of refractory AOSD patients.

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This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors, including pharmaceutic agencies.

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Concept: M.M., M.R.C., A.B.; design: M.R.C., D.Č.; supervision: B.A., M.M.; materials: M.M., A.B.; data collection and/or processing: D.Č., A.B., M.R.C.; analysis and/or interpretation: B.A.; literature search: M.R., D.Č.; writing manuscript: M.R., D.Č.; critical review: B.A.

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Correspondence to Mirna Reihl Crnogaj.

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Reihl Crnogaj, M., Čubelić, D., Babić, A. et al. Treatment of refractory adult onset Still’s disease with tocilizumab—a single centre experience and literature review. Rheumatol Int 40, 1317–1325 (2020).

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