Abstract
BACKGROUND: Infants with Kawasaki Disease (KD) have a higher risk of incomplete presentations, IVIG resistance, and coronary artery lesions (CALs). IL-1 plays a key role in the pathogenesis, highlighting its potential as a therapeutic target. OBJECTIVE: To report a multicenter Italian experience and review the literature on anakinra use in KD infants with CALs. METHODS: We retrospectively reviewed charts of patients aged ≤ 6 months treated with anakinra at four Italian centers between 2015 and 2024. A systematic Literature search was also conducted in PubMed, Scopus, Embase, and the Cochrane Library up to October 2024. RESULTS: Eight infants were included. The median age at diagnosis was 2.75 months. Six had incomplete KD. All were resistant to first-line treatment and all developed CALs, which were detected at a median of 9.5 days from fever onset. Anakinra was initiated at a median of 18 days from fever onset and 1.5 days after CALs detection. One patient received only subcutaneous anakinra. Seven infants underwent intravenous administration (median dose 8.5 mg/kg/day), four of whom received an initial bolus (median dose 2.75 mg/kg), and six were subsequently switched to subcutaneous dosing. Median total treatment duration was 22.5 days. CALs completely resolved in five patients, and improved in two. One treatment-related adverse event was reported. The literature review identified nine additional infants ≤ 6 months; seven showed systemic improvement and five had coronary improvement, and no adverse events were reported after anakinra treatment. CONCLUSION: Anakinra may be a promising and well-tolerated option for infants with KD and CALs, especially in IVIG-resistant or high-risk cases. While adverse events were unusual, further studies are needed to confirm its safety and efficacy. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12969-025-01143-x.