Treatment of resistant adult still disease with infliximab: a report of 2 cases.

Adult Still disease is characterized by high fever, arthritis, leukocytosis, hepatosplenomegaly, rash, and lymphadenopathy. It affects primarily young adults and is often disabling or even fatal. Many patients achieve long-term remissions, while others may develop a chronic illness refractory to multiple therapies. Polyarthritis or root joint involvement has been identified as a marker of chronic disease. No controlled studies on second-line agents have been published, but anecdotal studies suggest efficacy of a variety of agents including hydroxychloroquine, sulfasalazine, penicillamine, and methotrexate. Although these therapies have been used, optimal treatment of patients with resistant disease remains problematic. High levels of tumor necrosis factor have been noted, and recent report indicates etanercept may be an effective therapy. We report 2 cases of adult-onset Still disease resistant to NSAIDs, steroids, and methotrexate that have been successfully maintained on 5 mg/kg infliximab for 30 and 29 months, respectively, with improvement of signs and symptoms despite a reduction of steroid dosage. In patients with resistant disease, infliximab may be a safe, effective agent. Its use in this disease merits further study.