Wilson’s disease, the most common inherited disorder of copper metabolism, results from a failure of the copper excretory pathway. This leads to toxic accumulation of copper in the liver (link to section in table that deals with liver copper levels) and eventually other organs.1 The prevalence of Wilson’s disease in the general population is 1 per 40,000. The gold standard for diagnosis of Wilson’s disease is liver biopsy, although other tests can be a useful guide.2
The condition is effectively treated with a low copper diet and chelating agents that bind copper to facilitate its excretion from the body. Cuprimine® is the first-line chelating agent recommended for removal of excess copper in patients with Wilson’s disease.3
Cuprimine® is also indicated in the treatment of cystinuria and in patients with severe, active rheumatoid arthritis who have failed to respond to an adequate trial of conventional therapy.3
Important Safety Information
CUPRIMINE® (Penicillamine) is a chelating agent used in the treatment of Wilson's disease. It is also used to reduce cystine excretion in cystinuria and to treat patients with severe active rheumatoid arthritis unresponsive to conventional therapy. Physicians planning to use penicillamine should thoroughly familiarize themselves with its toxicity, special dosage considerations and therapeutic benefits. Penicillamine should never be used casually. Each patient should remain constantly under the close supervision of the physician. Patients should be warned to report promptly any symptoms suggesting toxicity. Penicillamine can cause fetal harm when administered to a pregnant woman, therefore except for the treatment of Wilson's disease or certain patients with cystinuria, use of penicillamine during pregnancy is contraindicated. Mothers on therapy with penicillamine should not nurse their infants. Patients with a history of penicillamine-related aplastic anemia or agranulocytosis should not be restarted on penicillamine. Because of its potential for causing renal damage, penicillamine should not be administered to rheumatoid arthritis patients with a history or other evidence of renal insufficiency. The use of penicillamine has been associated with fatalities due to certain diseases such as aplastic anemia, agranulocytosis, thrombocytopenia, Goodpasture's syndrome, and myasthenia gravis. Leucopenia and thrombocytopenia have been reported to occur up to five percent of patients during penicillamine therapy. Proteinuria and/or hematuria may develop during therapy and may be warning signs of membranous glomerulopathy which can progress to t nephritic syndrome. Intrahepatic cholestasis, toxic hepatitis and obliterative bronchiolitis have been reported. Onset of new neurological symptoms has been reported, or existing neurological symptoms have worsened. Most of the various forms of pemphigus have occurred during treatment with penicillamine. Some patients may experience a drug fever sometimes accompanied with a macular cutaneous eruption. Early and late rashes have occurred. Certain patients may develop a positive antinuclear antibody (ANA) test and some of these may show a lupus erythematosus-like syndrome. Some patients may develop oral ulcerations. Hypogeusia has been reported in some patients. Penicillamine should not be used in patients who are receiving concurrently gold therapy, antimalarial or cytotoxic drugs, oxyphenbutazone or phenylbutazone.
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Reference: 1. Gaffney D, Fell GS, O'Reilly St. J. Wilson's disease: acute and presymptomatic laboratory diagnosis and monitoring. J Clin Pathol. 2000;53:807-12.
2. Brewer GJ. Wilson's disease. In: Kasper DL, Fauci AS, Longo DL, eds. Harrison's Principles of Internal Medicine. New York. 16th ed. McGraw-Hill. 2005:2313-15.
3. Cuprimine package insert. Lawrenceville, NJ: Aton Pharma; 2007.