Formulary
Penicillamine: Indications, Dosing, Side Effects and Interactions
Penicillamine aids the elimination of copper ions in Wilson's disease (hepatolenticular degeneration).
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Penicillamine: Indications, Dosing, Side Effects and Interactions
Penicillamine aids the elimination of copper ions in Wilson's disease (hepatolenticular degeneration).
Drug action
Penicillamine aids the elimination of copper ions in Wilson's disease (hepatolenticular degeneration).
Indications and dose
**Severe active rheumatoid arthritis (administered on expert advice)**
- **Adult** (By Mouth): 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** (By Mouth): 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**
- **Adult** (By Mouth): 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** (By Mouth): 20 mg/kg daily in divided doses, adjusted according to response.
**Autoimmune hepatitis (used rarely; after disease controlled with corticosteroids)**
- **Adult** (By Mouth): Initially 500 mg daily in divided doses, to be increased slowly over 3 months; maintenance 1.25 g daily.
**Cystinuria, therapeutic**
- **Adult** (By Mouth): 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**
- **Adult** (By Mouth): 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** (By Mouth): Minimum dose to maintain urinary cystine below 200 mg/litre is recommended.
**Wilson's disease for penicillamine**
- **Child 1 month-11 years** (By mouth): 20 mg/kg daily in 2-3 divided doses, to be taken 1 hour before food; maximum 2 g per day.
- **Child 12-17 years** (By mouth): Initially 20 mg/kg daily in 2-3 divided doses. maintenance 0.75-1 g daily, to be taken 1 hour before food; maximum 2 g per day.
**Cystinuria for penicillamine**
- **Child** (By mouth): 20-30 mg/kg daily in 2-3 divided doses, lower doses may be used initially and increased gradually, doses to be adjusted to maintain 24-hour urinary cystine below 1 mmol/litre, maintain adequate fluid intake, to be taken 1 hour before food; maximum 3 g per day.
Cautions
Neurological involvement in Wilson's disease
Contraindications
Lupus erythematosus
Side effects
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; pneumonitis; polymyositis; pulmonary haemorrhage; rash (consider dose reduction); respiratory tract inflammation; 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.
Interactions
**Other interactions (5):**
- Concomitant use of iron or antacids: oral absorption of penicillamine may be reduced by concomitant administration of iron or antacid (see section 4.4).
- Concomitant use of digoxin: oral absorption of digoxin may be reduced by concomitant administration of penicillamine (see section 4.4).
- Concomitant use of clozapine: penicillamine may potentiate the blood dyscrasias seen with clozapine (see section 4.4).
- Concomitant use of zinc: oral absorption of penicillamine may be reduced by concomitant administration of zinc; absorption of zinc may also be reduced by penicillamine.
- Pyridoxine daily may be given to patients on long term therapy, especially if they are on a restricted diet, since penicillamine increases the requirement for this vitamin (see section 4.4).
Pregnancy
Fetal abnormalities reported rarely; avoid if possible.
Breast feeding
Manufacturer advises avoid unless potential benefit outweighs risk-no information available.
Renal impairment
Caution in mild impairment; avoid in moderate to severe impairment. M Dose adjustments Dose reduction may be required in mild impairment depending on indication (consult product literature). M
Medicinal forms
Solution
Clinical governance
**Author:** MedNext Clinical Team. **Clinical reviewer:** Dr Shameer Deen, MBBS, MS, MRCS. **Sources:** BNF, Indian Pharmacopoeia, CIMS India. **Correction:** Report errors at support@mednext.academy.
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