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Juvenile idiopathic arthritis is a chronic inflammatory disease with uncertain outcome. Patients may suffer from severe joint damage leading to mutilations as well as from extra-articular manifestations. Prognosis is variable and depends in part on the number of affected joints and the occurrence of extra-articular manifestations. Treatment regimes should take this into account. Pharmacomedical treatment strategies include the application of nonsteroidal antirheumatics, corticosteroids, sulfasalazine, and immunosuppressive substances. However, of the latter only methotrexate has been shown in controlled trials to be effective. Other immunosuppressive drugs such as azathioprine, cyclosporine A, and leflunomide have not yet been investigated sufficiently. In addition, there is no scientific basis for the application of gold salts or (hydroxy)chloroquine. New therapeutic biologic agents, notably the tumor necrosis factor inhibitors, have achieved dramatic improvements also in patients with severe, as yet intractable disease. However, until now randomized, placebo-controlled trials have been performed for etanercept only. Dramatic improvement was accompanied by low toxicity. Infections as well as the development of autoimmune diseases have been shown to be the major potential side effects. Long-term toxicity still has to be evaluated. Treatment with other biopharmaceuticals such as infliximab, IL-1Ra, anti-IL-6 receptor antibodies, and further cytokine antagonists remains experimental.