Abstract
BACKGROUND: Anakinra, an interleukin-1 receptor antagonist, is widely used to manage systemic juvenile idiopathic arthritis (sJIA) and related inflammatory conditions. While generally well tolerated, rare cases of drug-induced liver injury (DILI) have been reported. METHODS: In addition to a literature review, we present a pediatric case of severe DILI attributed to anakinra. The case is of a 14-year-old male with sJIA complicated by macrophage activation syndrome (MAS) who developed DILI following the initiation of anakrina. RESULTS: Anakrina was started after initial corticosteroid treatment. Following dose escalation, he developed a marked elevation in liver enzymes and cholestasis. Extensive workup, including a liver biopsy, excluded other causes and supported a diagnosis of anakinra-induced DILI. Discontinuation of anakinra led to the gradual normalization of liver function. Subsequent treatment with cyclosporine and tocilizumab was well tolerated. CONCLUSIONS: Differentiating the liver enzyme elevation to MAS versus DILI is challenging but critical, as management strategies differ. Anakinra-induced liver injury appears to be an idiosyncratic, immune-mediated phenomenon that resolves on drug withdrawal. Our case highlights the importance of monitoring liver function during anakinra therapy and the role of liver biopsy in complex cases.