Guidelines Provide New Insights Into Systemic JIA Treatment
A MEETING SPONSORED BY NEW YORK UNIVERSITY
Although it was not discussed in the Recommendations, in his talk Dr. Cron said that anakinra is "revolutionizing" the treatment of macrophage activation syndrome (MAS) in systemic JIA (Rheumatology 2011;50:417-9) Dr. Cron reported that he has used anakinra as initial therapy to treat two children with MAS, and he found dramatic reductions in ferritin levels and liver enzymes within 2 days of initiation.
Systemic JIA is a relatively rare disease, with a prevalence of about 1 in 10,000. It has multiple systemic manifestations, including a high fever (which follows a unique pattern: typically once-daily, late-afternoon spikes in 30% of patients at presentation), rash, hepatosplenomegaly, lymphadenopathy, pericarditis, pleural effusion, pulmonary vasculitis, and even CNS stroke or seizure. Stroke or seizure might be related to MAS, a widespread coagulopathy that occurs in about 50% of patients and can be fatal.
Another common finding is asymmetry of the jaw. According to Dr. Cron, up to 80% of children with all forms of arthritis, including systemic JIA, can have arthritis of the temporomandibular joint (TMJ). This is often a subtle finding that can be overlooked until significant erosion has occurred. However, early TMJ arthritis can be detected with magnetic resonance screening.
Dr. Cron is a consultant for Genentech.
