Wilson's disease: clinical management and therapy

This review focuses on the treatment and management of patients with Wilson's disease. A central feature of management is choice of anticopper drug or drugs for various stages and classes of disease. Physicians should no longer rely on penicillamine as their major choice for treating this disease. In fact, our recommendation is that penicillamine be rarely used. For initial treatment of the hepatic failure presentation, we recommend a combination of trientine and zinc. For initial treatment of the neurologic/psychiatric presentation, we recommend tetrathiomolybdate and zinc. If tetrathiomolybdate is not available our second choice is zinc alone. For maintenance therapy (usually 2--4 months after initial therapy), for presymptomatic patients, and for pregnant patients, zinc is recommended with trientine as second choice. Monitoring recommendations for both efficacy and safety for these various anticopper drugs are provided. Hepatic transplantation should be reserved for only patients with severe liver failure, and never used for neurologic indications. Information on how to triage liver failure patients is provided. Other aspects of management, including diet, drinking water, physical therapy, and concomitant medical therapy, as well as prognosis and long term risks are also discussed. © 2004 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.