Penicillamine

INDICATIONS

Penicillamine capsules are indicated in the treatment of Wilson's disease, cystinuria, and in patients with severe, active rheumatoid arthritis who have failed to respond to an adequate trial of conventional therapy. Available evidence suggests that penicillamine capsules are not of value in ankylosing spondylitis.

W il son’s Disease — Wilson’s disease (hepatolenticular degeneration) occurs in individuals who have inherited an autosomal recessive defect that leads to an accumulation of copper far in excess of metabolic requirements. The excess copper is deposited in several organs and tissues, and eventually produces pathological effects primarily in the liver, where damage progresses to postnecrotic cirrhosis, and in the brain, where degeneration is widespread.

Copper is also deposited as characteristic, asymptomatic, golden-brown Kayser-Fleischer rings in the corneas of all patients with cerebral symptomatology and some patients who are either asymptomatic or manifest only hepatic symptomatology.

1. Severe active rheumatoid arthritis including juvenile forms. 2. Wilson’s disease (hepatolenticular degeneration) in adults and children (0 to 18 years). 3. Cystinuria – dissolution and prevention of cystine stones in adults and children (0 to 18 years).

4. Lead poisoning in adults and children (0 to 18 years). 5. Chronic active hepatitis in adults.

29 Product Monograph - CUPRIMINE® Page 3 of 31 PrCUPRIMINE® Penicillamine Capsules, USP PART I: HEALTH PROFESSIONAL INFORMATION SUMARY PRODUCT INFORMATION Route of Administration Dosage Form / Strength Clinically Relevant Nonmedicinal Ingredients oral Capsules 250 mg Lactose For a complete listing see Dosage Forms, Composition and Packaging section.

INDICATIONS AND CLINICAL USE CUPRIMINE (penicillamine capsules, USP) is indicated for:  treatment of Wilson's disease  chronic lead poisoning  cystinuria, and  In patients with severe, active rheumatoid arthritis who have failed to respond to an adequate trial of conventional therapy.

Available evidence suggests that CUPRIMINE is not of value in ankylosing spondylitis. Because of the severe toxicity of this agent, penicillamine should never be used casually. Wilson's Disease Treatment has two objectives: (1) to minimize dietary intake and absorption of copper.

(2) to promote excretion of copper deposited in tissues. For the second objective, a copper chelating agent is used. Penicillamine is the only one of these agents that is orally effective. In symptomatic patients, this treatment usually produces marked neurologic improvement, fading of Kayser-Fleisher rings, and gradual amelioration of hepatic dysfunction and psychic disturbances.