Anakinra in the management of adult-onset still’s disease: a single-center experience

Abstract

Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disorder of unknown etiology characterized by systemic inflammation, high fever, salmon-colored skin rash, arthralgia, and arthritis. Patients with AOSD may also present with elevated inflammatory markers, hyperferritinemia, anemia, leukocytosis, hepatosplenomegaly, and lymphadenopathy. Glucocorticoids and biological disease-modifying anti-rheumatic drugs, including the anti-interleukin-1 agent anakinra, are used in the management of AOSD. This retrospective single-center study included patients with AOSD who were registered at our tertiary center, and received anakinra treatment. The primary outcome of our study was the proportion of patients who achieved complete remission of disease-related clinical and laboratory complications. The glucocorticoid treatment profiles of the included patients before and after anakinra treatment were also analyzed. The occurrence of serious and non-serious adverse events was recorded to analyze the safety profile of anakinra. Thirty-four patients with AOSD, including 25 females (73.5%), were enrolled in the study. Twelve patients (35.3%) achieved complete remission and 14 patients (41.2%) achieved partial remission after anakinra treatment. Eight patients (23.5%) did not response to anakinra. Anakinra significantly decreased the number of patients receiving glucocorticoid treatment [33 (97%) vs. 22 (64.7%), p < 0.001] and the mean daily glucocorticoid dose [19 ± 13.5 mg vs. 4.6 ± 5.8 mg, p < 0.001]. Mild adverse events occurred in 11 patients (32.3%) with injection site reactions being the most common. One patient (2.9%) was diagnosed with tuberculosis within the treatment period. Anakinra is an effective and generally safe option for biological treatment initiation in the management of AOSD.