Wilson's disease medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Recommendations for the treatment of Wilson's Disease (DO NOT EDIT)
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Treatment in Specific Clinical Situations
Treatment Targets and Monitoring of Treatment
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Medical therapy
Various treatments are available for Wilson's disease. Some increase the removal of copper from the body, while others prevent the absorption of copper from the diet. In general, a diet low in copper-containing foods (mushrooms, nuts,chocolate, dried fruit, liver, and shellfish) is recommended.
Generally, penicillamine is the first treatment used. This binds copper (chelation) and leads to excretion of copper in the urine. Hence, monitoring of the amount of copper in the urine can be done to ensure a sufficiently high dose is taken. Penicillamine is not without problems: about 20% of patients experience a side effect or complicaton of penicillamine treatment, such as drug-induced lupus (causing joint pains and a skin rash) or myasthenia (a nerve condition leading to muscle weakness). In those who presented with neurological symptoms, almost half experience a paradoxical worsening in their symptoms. While this phenomenon is also observed in other treatments for Wilson's, it is usually taken as an indication for discontinuing penicillamine and commencing second-line treatment. Patients intolerant to penicillamine may instead be commenced on trientine hydrochloride, which also has chelating properties. Some recommend trientine as first-line treatment, but experience with penicillamine is more extensive. A further agent with known activity in Wilson's disease is tetrathiomolybdate. This is still regarded as experimental, although some studies have shown a beneficial effect.
Once all results have returned to normal, zinc (usually in the form of zinc acetate) may be used instead of chelators to maintain stable copper levels in the body. Zinc stimulates metallothionein, a protein in gut cells that binds copper and prevents their absorption and transport to the liver. Zinc therapy is continued unless symptoms recur, or if the urinary excretion of copper increases.
In rare cases where none of the oral treatments are effective, especially in severe neurological disease, dimercaprol (British anti-Lewisite) is still occasionally necessary. This treatment is injected intermuscularly (into a muscle) every few weeks, and has a number of unpleasant side effects such as pain.[1]
People who are asymptomatic (for instance those diagnosed through family screening or only as a result of abnormal test results) are generally treated, as the copper accumulation may cause long-term damage in the future. It is unclear whether these people are best treated with penicillamine or zinc acetate.