Drug Action
Penicillamine aids the elimination of copper ions in Wilson’s disease (hepatolenticular degeneration).
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Drug reference
Penicillamine aids the elimination of copper ions in Wilson’s disease (hepatolenticular degeneration).
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Severe active rheumatoid arthritis (administered on expert advice) ▶BY MOUTH ▶Adult: Initially 125–250 mg daily for 1 month, then increased in steps of 125–250 mg, at intervals of not less than 4 weeks; maintenance 500–750 mg daily in divided doses, then reduced in steps of 125–250 mg every 12 weeks, dose reduction attempted only if remission sustained for 6 months; maximum 1.5 g per day ▶Elderly: Initially up to 125 mg daily for 1 month, then increased in steps of up to 125 mg, at intervals of at least 4 weeks; maximum 1 g per day Wilson’s disease ▶BY MOUTH ▶Adult: 1.5–2 g daily in divided doses, adjusted according to response, to be taken before food; maintenance 0.75–1 g daily, a dose of 2 g daily should not be continued for more than one year; maximum 2 g per day ▶Elderly: 20 mg/kg daily in divided doses, adjusted according to response Autoimmune hepatitis (used rarely; after disease controlled with corticosteroids) ▶BY MOUTH ▶Adult: Initially 500 mg daily in divided doses, to be increased slowly over 3 months; maintenance 1.25 g daily Cystinuria, therapeutic ▶BY MOUTH ▶Adult: 1–3 g daily in divided doses, to be adjusted to maintain urinary cystine below 200 mg/litre, to be taken before food Cystinuria, prophylactic ▶BY MOUTH ▶Adult: 0.5–1 g daily, maintain urinary cystine below 300 mg/litre and adequate fluid intake (at least 3 litres daily), to be taken at bedtime ▶Elderly: Minimum dose to maintain urinary cystine below 200 mg/litre is recommended
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Lupus erythematosus
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Neurological involvement in Wilson’s disease
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Patients who are hypersensitive to penicillin may react rarely to penicillamine.
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→Appendix 1: penicillamine
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▶Common or very common Proteinuria. thrombocytopenia ▶Rare or very rare Alopecia. breast enlargement (males and females). connective tissue disorders. haematuria (discontinue immediately if cause unknown). hypersensitivity. oral disorders. skin reactions ▶Frequency not known Agranulocytosis. aplastic anaemia. appetite decreased. fever. glomerulonephritis. Goodpasture’s syndrome. haemolytic anaemia. increased risk of infection. jaundice cholestatic. leucopenia. lupus- like syndrome. myasthenia gravis. nausea. nephrotic syndrome. neurological deterioration in Wilson’s Disease. neutropenia. pancreatitis. polymyositis. pulmonary haemorrhage. rash (consider dose reduction). respiratory disorders. Stevens-Johnson syndrome. taste loss (mineral supplements not recommended). vomiting. yellow nail syndrome SIDE-EFFECTS, FURTHER INFORMATION Proteinuria occurs in up to 30% of patients—can be a sign of immune- mediated nephropathy. Discontinue immediately if nephrotoxicity occurs. Nausea and rash more common early in treatment if full dose used from initiation. Delayed rash can occur after months or years of treatment—manufacturer advises reduce dose.
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Fetal abnormalities reported rarely; avoid if possible.
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Manufacturer advises avoid unless potential benefit outweighs risk—no information available.
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Dose adjustments Reduce dose and monitor renal function or avoid (consult product literature).
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▶Consider withdrawal if platelet count falls below 120 000/mm3 or white blood cells below 2500/mm3 or if 3 successive falls within reference range (can restart at reduced dose when counts return to within reference range but permanent withdrawal necessary if recurrence of leucopenia or thrombocytopenia). ▶Blood counts, including platelets, and urine examinations should be carried out before starting treatment and then every 1 or 2 weeks for the first 2 months then every 4 weeks to detect blood disorders and proteinuria (they should also be carried out in the week after any dose increase). ▶A reduction in platelet count calls for discontinuation with subsequent re-introduction at a lower dosage and then, if possible, gradual increase. ▶Longer intervals may be adequate in cystinuria and Wilson’s disease.
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There can be variation in the licensing of different medicines containing the same drug. Forms available from special-order manufacturers include: oral solution Tablet CAUTIONARY AND ADVISORY LABELS 6, 22 ▶Penicillamine (Non-proprietary) Penicillamine 125 mg Penicillamine 125mg tablets | 56 tablet P £59.61 DT = £51.79 Penicillamine 250 mg Penicillamine 250mg tablets | 56 tablet P £113.70 DT = £104.23
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Counselling on the symptoms of blood disorders is advised. Warn patient and carers to tell doctor immediately if sore throat, fever, infection, non-specific illness, unexplained bleeding and bruising, purpura, mouth ulcers, or rashes develop.
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Initially 125–250 mg daily for 1 month, then
First-line fragment. Read the complete Indications and Dose section above.
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1.5–2 g daily in divided doses, adjusted
First-line fragment. Read the complete Indications and Dose section above.
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Initially 500 mg daily in divided doses, to be
First-line fragment. Read the complete Indications and Dose section above.
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1–3 g daily in divided doses, to be adjusted to
First-line fragment. Read the complete Indications and Dose section above.
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0.5–1 g daily, maintain urinary cystine below
First-line fragment. Read the complete Indications and Dose section above.
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