Tumor necrosis factor blockade in the management of children with orphan diseases.

Tumor necrosis factor (TNF) blockade has been used successfully to treat a number of rheumatic disorders that have a substantial burden of illness. In children, the TNF antagonists are used mainly for the treatment of juvenile idiopathic arthritis (JIA). There are, however, a variety of rare systemic inflammatory diseases, in which TNF blockade appears promising. Preliminary data in adults suggest that several forms of vasculitis appear to be responsive to TNF antagonists-Behcet's disease, polyarteritis nodosa, Wegener granulomatosis, among others. Some of them respond better to infliximab, a chimeric monoclonal anti-TNF antibody, than to etanercept, a recombinant p75 TNF receptor. We describe our limited experience with infliximab in the treatment of three children with rare vasculitic conditions.