Introduction
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 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. Syprine® is a chelating agent indicated for treatment of patients with Wilson's disease who are intolerant of the first-line treatment, penicillamine. 3
Indications and Usage
SYPRINE is indicated in the treatment of patients with Wilson's disease who are intolerant of penicillamine. Clinical experience with SYPRINE is limited and alternate dosing regimens have not been well-characterized; all endpoints in determining an individual patient's dose have not been well defined. SYPRINE and penicillamine cannot be considered interchangeable. SYPRINE should be used when continued treatment with penicillamine is no longer possible because of intolerable or life endangering side effects.
Unlike penicillamine, SYPRINE is not recommended in cystinuria or rheumatoid arthritis. The absence of a sulfhydryl moiety renders it incapable of binding cystine and, therefore, it is of no use in cystinuria. In 15 patients with rheumatoid arthritis, SYPRINE was reported not to be effective in improving any clinical or biochemical parameter after 12 weeks of treatment. SYPRINE is not indicated for treatment of biliary cirrhosis.
Important Safety Information
SYPRINE® is contraindicated in patients hypersensitive to SYPRINE, or any components of the formulation. Patients should be observed closely for signs of possible hypersensitivity. Patients receiving SYPRINE should remain under regular medical supervision throughout the period of drug administration. Patients (especially women) should be closely monitored for evidence of iron deficiency anemia. Clinical experience is limited.
SYPRINE must be taken on an empty stomach, at least one hour before meals or two hours after meals and at least one hour apart from any other drug, food, or milk. The capsules should be swallowed whole with water and should not be opened or chewed.
For the first month of treatment, the patient should have his temperature taken nightly, and report any symptom such as fever or skin eruption.
The following adverse reactions have been reported from a clinical study: iron deficiency and systemic lupus erythematosus. In addition, dystonia, muscular spasm and myasthenia gravis have been reported in marketed use.
In general, mineral supplements should not be given since they may block the absorption of SYPRINE.
You are encouraged to report negative side effects of prescription drugs to FDA. Visit www.FDA.gov/medwatch or call 1-800-FDA-1088.
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. http://www.syprine.com/pdfs/Syprine_PI.pdf; Syprine package insert. Lawrenceville, NJ Aton Pharma; 2007.
