Importance of the field: Systemic juvenile idiopathic arthritis (sJIA) is a debilitating childhood disease presenting with fever, rash and arthritis. Current therapy consisting mainly of corticosteroids, NSAIDs and methotrexate, can be inefficient and is often accompanied by many serious adverse events. Although an IL-1 receptor antagonist (anakinra) seems to be very efficient in case series, it is not registered for use in sJIA. Pediatric rheumatologists all over the world are having a hard time to convince insurance companies to approve off-label use of this drug for their sJIA patients.
Areas covered in this review: Using the MeSH terms 'Arthritis Juvenile Rheumatoid' and 'Interleukin 1 Receptor Antagonist Protein', we searched MEDLINE, EMBASE and reference lists of selected articles. This review is largely based on manuscripts from 2005 to 2010 and abstracts presented at the major (pediatric) rheumatology congresses.
What the reader will gain: This review summarizes the data of 140 children with sJIA treated with anakinra and enables an understanding in the benefit-risk profile of anakinra in sJIA patients.
Take home message: Anakinra has shown to be a very efficient and quick acting medicine in reducing symptoms even in therapy-resistant sJIA patients with typically poor outcomes.