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Penicillamine

Pronunciation: PEN-ih-SILL-ah-meen
Class: Cystine-depleting agent

Trade Names

Cuprimine
- Capsules 250 mg

Depen
- Tablets, titratable 250 mg

Pharmacology

Unknown; however, appears to suppress disease activity.

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Indications and Usage

Treatment of Wilson disease; cystinuria; and severe, active rheumatoid arthritis.

Contraindications

Pregnancy (except in treatment of Wilson disease or certain cases of cystinuria); penicillamine-related aplastic anemia or agranulocytosis; rheumatoid arthritis patients with a history of renal insufficiency; breast-feeding.

Dosage and Administration

Cystinuria
Adults

PO Initially, 250 mg/day, and increasing gradually to the requisite amount. Range 1 to 4 g/day. Daily dose should be divided into 4 doses.

Children

PO 30 mg/kg/day divided into 4 doses. If 4 equal doses are not feasible, give the larger portion at bedtime.

Rheumatoid Arthritis
Adults

PO Start with a single daily dose of 125 to 250 mg/day. The dose may be increased at 1- to 3-mo intervals by 125 to 250 mg/day, as patient response and tolerance indicate. Continue dosage associated with satisfactory remission. If there is no improvement or no signs of potentially serious toxicity after 2 to 3 mo of treatment with 500 to 750 mg/day, increases of 250 mg/day at 2- to 3-mo intervals may be continued until satisfactory remission or signs of toxicity develop. If there is no discernible improvement after 3 or 4 mo of treatment with 1,000 to 1,500 mg/day, penicillamine should be discontinued.

Maintenance therapy

Many patients respond satisfactorily to dosage within the 500 to 750 mg/day range.

Duration

If a patient has been in remission for 6 mo or more, a gradual, stepwise dosage reduction in decrements of 125 or 250 mg at approximate 3-mo intervals may be attempted.

Wilson Disease
Adults

PO 0.25 mg to 2 g/day. Optimal dosage can be determined by measurement of urinary copper excretion and determination of free copper in the serum.

Storage/Stability

Store between 59° and 86°F. Protect from moisture.

Drug Interactions

Aluminum salts (eg, aluminum carbonate, sucralfate)

GI absorption of penicillamine may be reduced.

Adverse Reactions

CNS

Peripheral sensory and motor neuropathies (including Guillain-Barré syndrome); psychic disturbances; mental disorders; agitation; anxiety.

Dermatologic

Urticaria; exfoliative dermatitis; alopecia; failing hair; lichen planus; toxic epidermal necrolysis; cutaneous macular atrophy; increased skin friability; excessive wrinkling; development of small white papules at venipuncture and surgical sites; yellow nail syndrome.

EENT

Tinnitus; optic neuritis; visual disturbances.

GI

Anorexia, epigastric pain, nausea, vomiting, diarrhea (17%); diminished taste perception (12%); peptic ulcers; pancreatitis; oral ulcers.

Endocrine

Thyroiditis.

Hematologic

Thrombocytopenia (4%); leukopenia (2%); agranulocytosis; aplastic anemia; sideroblastic anemia; hemolytic anemia; thrombotic thrombocytopenic purpura; red cell aplasia; monocytosis; leukocytosis; eosinophilia; thrombocytosis.

Hepatic

Intrahepatic cholestasis; toxic hepatitis.

Musculoskeletal

Migratory polyarthralgia; myasthenia gravis; dystonia.

Renal

Proteinuria (6%); hematuria; nephrotic syndrome; renal failure.

Respiratory

Allergic alveolitis, obliterative bronchiolitis; interstitial pneumonitis; pulmonary fibrosis; bronchial asthma.

Miscellaneous

Allergy, including generalized pruritus with early and late rashes (5%); pemphigus; drug eruptions (accompanied by fever, arthralgia, lymphadenopathy); lupus erythematosus-like syndrome; thrombophlebitis; hyperpyrexia; polymyositis; dermatomyositis; mammary hyperplasia; elastosis perforans serpiginosa; Goodpasture syndrome; vasculitis (including fatal renal vasculitis).

Precautions

Warnings

Physicians should be thoroughly familiar with penicillamine toxicity, special dosage considerations, and therapeutic benefits. Patients should promptly report any symptoms of toxicity.


Pregnancy

Contraindicated in pregnancy.

Lactation

Breast-feeding is contraindicated.

Children

Safety and efficacy not established in children with juvenile rheumatoid arthritis.

Allergy

Skin and mucous membranes should be observed for allergic reactions.

Pemphigus

Pemphigus vulgaris or pemphigus foliaceus may be late complications of therapy.

Severe reactions

Penicillamine therapy has a high incidence of adverse reactions, some of which are potentially fatal. Treatment has been associated with aplastic anemia, agranulocytosis, thrombocytopenia, Goodpasture syndrome, myasthenia gravis, proteinuria, hematuria, and obliterative bronchiolitis.

Copyright © 2009 Wolters Kluwer Health.

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