Ab1406 Anakinra in the Management of Adult-Onset Still’s Disease: A Single-Center Experience

Background: 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, disease-modifying anti-rheumatic drugs (DMARDs), and biological drugs, including the anti-IL-1 agent anakinra, are used in the management of AOSD. Objectives: The main purpose of this study was to evaluate the effectiveness of anakinra in suppressing disease activity, tapering glucocorticoids, and preventing undesired metabolic effects of glucocorticoids in patients with AOSD. This study also aimed to assess the tolerability of the anakinra treatment. Methods: In this retrospective single-center study, we included patients who were registered at our tertiary center with a diagnosis of AOSD according to the Yamaguchi criteria and had received treatment with anakinra. Patients were allowed to receive DMARDs or glucocorticoids prior to or concomitant with anakinra treatment. The primary outcome of our study was the proportion of patients who achieved complete remission of disease-related complications, including MAS, fever, arthralgia, arthritis, rash, hepatomegaly, splenomegaly, pericarditis, pleuritis, peritonitis, lymphadenopathy, anemia, hyperferritinemia, leukocytosis, elevated inflammatory markers (CRP and ESR), and elevated liver enzymes (AST and ALT) after anakinra treatment. Partial remission was defined as ≥ 50% improvement in the aforementioned findings. Any other outcome was considered a treatment failure. We analyzed the use and daily dose of glucocorticoids before and after anakinra treatment. As an aspect of glucocorticoid toxicity, we evaluated changes in body mass index (BMI) after anakinra treatment. Each patient was called for a physical examination, online methods were used when a physical examination was not performed. Other data sources included patient files and digital institutional records. Results: Our study included 29 patients with AOSD, 21 (72.4 %) of whom were female. The mean age and mean disease duration were 39.17 ± 13.63 and 6.59 ± 3.32 years, respectively. The mean duration of anakinra treatment was 22.35 ± 26.49 months. Eleven patients (37.9 %) achieved complete remission after anakinra treatment. Partial remission was achieved in 10 (34.5 %) patients, and treatment failed in 8 (27.6 %) patients. Anakinra significantly reduced the proportion of patients who experienced fever, arthritis, arthralgia, rash, lymphadenopathy, and elevated inflammatory markers. Anakinra was also associated with a significant improvement in anemia and leukocytosis (Figure 1). Twenty-seven patients (93.1 %) received glucocorticoids prior to the first application of anakinra. This number reduced to 20 (69 %) after treatment, indicating a significant reduction (p=0.04). Among patients who received anakinra the mean daily glucocorticoid dose decreased from 17.62 ± 13.2 mg to 4.79 ± 5.1 mg (p<0.001). However, the mean BMI increased from 25.23 ± 5.25 to 27.15 ± 6.25 (p=0.016). Anakinra demonstrated significant improvements in patient-reported disease activity scores and acute phase responses (Figure 2). Twenty patients (69%) were not receiving anakinra at the time the study was conducted. The most common reasons for treatment discontinuation were injection site reactions and a lack of efficacy. Mild adverse events occurred in 10 patients (34.5 %), with injection site reactions being the most frequent. No serious adverse events were observed during anakinra treatment. Conclusion: Anakinra was effective in resolving disease-related complications, suppressing inflammatory markers, and tapering glucocorticoids, with a low adverse event profile in patients with AOSD. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: None declared.