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

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.

Efficacy and Safety of Biological Drugs in Still's Disease: A Systematic Review and Network Meta-Analysis of Randomized Controlled Trials.

OBJECTIVES: Still's disease is a rare autoinflammatory disorder characterized by systemic inflammation, fever, rash, and arthritis. The term "Still's disease" covers the pediatric subtype systemic Juvenile Idiopathic Arthritis (sJIA) and adult-onset Still's disease (AOSD), which affects adults. Biological drugs, including anti-interleukin-1 agents anakinra, canakinumab, rilonacept, and the interleukin-6 antagonist tocilizumab, are used in the management of Still's disease. METHODS: We conducted a systematic review and network meta-analysis of randomized controlled trials, and the study protocol was registered in PROSPERO (CRD42023450442). MEDLINE, EMBASE, and CENTRAL were screened from inception until September 17, 2023. We included patients with Still's disease who received placebo or biological drugs: anakinra, canakinumab, rilonacept, or tocilizumab. The primary efficacy and safety outcomes were achievement of ACR50 response and occurrence of serious adverse events, respectively. The interventions were ranked using rankograms and SUCRA values. RESULTS: Nine trials with 430 patients were included. All biological drugs were associated with greater odds of ACR50 response compared with placebo. There was no statistically significant association between biological drugs and serious adverse events. The multivariate meta-analysis found no difference between biological drugs. As per SUCRA rankings, anakinra was the most effective and safe option with respect to ACR50 response and occurrence of serious adverse events. CONCLUSION: This is the first systematic review and meta-analysis to assess the efficacy and safety of biological drugs in pediatric and adult patients with Still's disease. Biological drugs were effective in achieving ACR response and demonstrated a low adverse event profile in the management of Still's disease.

Anakinra in idiopathic recurrent pericarditis: a comprehensive case series and literature review.

OBJECTIVE: Idiopathic recurrent pericarditis (IRP) is defined by recurring episodes of pericardial inflammation without a known cause. This study investigates the safety and efficacy of anakinra, an interleukin­1 inhibitor, as a successful therapy for IRP in cases resistant to conventional treatment. METHODS: A retrospective evaluation of patients treated at our autoinflammatory center between 2011 and 2023 was conducted. Patient files were examined for demographic, clinical, and treatment response data, including nonsteroid anti-inflammatory drugs (NSAIDs), corticosteroids, and colchicine. Monogenic autoinflammatory disease screening was performed for Mediterranean Fever (MEFV), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), mevalonate kinase (MVK), nucleotide-binding domain, leucine-rich-containing family, pyrin domain-containing-3 (NLRP3), and nucleotide-binding oligomerization domain-containing protein 2 (NOD2). Patients who experienced multiple episodes of pericarditis were diagnosed with recurrent pericarditis. The study evaluated anakinra treatment in IRP patients unresponsive to conventional therapy. RESULTS: The study included 21 participants, 9 (42.9%) female and 12 (57.1%) male. The average age of the participants was 43.1 ± 16.5 years. The MEFV mutation analysis revealed that 2 (9.5%) had a mutation in exon 10 and 4 (19.0%) had one in exon 2. Out of the 16 cases, 15 successfully discontinued steroid treatment. Four patients (19.0%) experienced injection site reactions. C­reactive protein (CRP) levels were measured at an average of 196 ± 67.8 mg/l before and 2.6 ± 3.15 mg/l after anakinra treatment. CONCLUSION: In conclusion, the study adds to the growing evidence for the efficacy of interleukin-1 inhibitors, such as anakinra, as a promising treatment modality for IRP in cases resistant to conventional treatment.