Dosing and uses of Cuprimine, Depen (penicillamine)
Adult dosage forms and strengths
tablet
- 250mg
capsule
- 125mg
- 250mg
Wilson's Disease
250 mg PO QID; dosage range 500-1500 mg/day
Pregnancy: Not to exceed 500-750 mg/day
Planned cesarean section: Reduce dose to 250 mg/day for the last 6 weeks of pregnancy and postoperatively until wound healing completed
Dosing considerations
- Adjust dose to achieve urinary copper excretion of 0.5-1 mg/day
- Free copper levels in serum: Maintain at <10 mcg/dL
Arsenic Poisoning
100 mg/kg/day PO divided q6hr x5 days
If used for chronic arsenic poisoning, endpoint: 24 hours urinary arsenic <50 mcg/L
Rheumatoid Arthritis
Initial: 125-250 mg/day PO
Maintenance: May be increased by 125-250 mg/day q1-3Months up to 500-750 mg/day, may be increased further if no response and patient tolerates
Lead Poisoning
1-1.5 g qDay PO or divided BID-TID x1-6 months
Administration
Usually given with 10-25 mg/day pyridoxine
Take on empty stomach; last dose given at least 3 hr after evening meaL
Other Indications & Uses
Cystinuria
Off-label: Lead toxicity (used following Ca-EDTA or dimercaprol; succimer preferred); copper, mercury, arsenic poisoning (chronic-only if BAL/DMSA unavailable); primary biliary cirrhosis
Pediatric dosage forms and strengths
tablet
- 250mg
capsule
- 125mg
- 250mg
Lead Poisoning
Considered 3rd-line therapy
20-40 mg/kg/day PO divided q8hr
Wilson's Disease
20 mg/kg/day PO divided q12hr
Cystinuria
30 mg/kg/day PO divided BID/QID; not to exceed 1 g/day
Juvenile Rheumatoid Arthritis
1st step (2 months): 5 mg/kg PO qDay x2 months
2nd step (4 months): 10 mg/kg PO qDay x4 months
Administration
Usually given with 10-25 mg/day pyridoxine
Take on empty stomach; last dose given at least 3 hours after evening meaL
Cystinuria: drink plenty of fluids
Cuprimine, Depen (penicillamine) adverse (side) effects
>10%
Worsening neurologic symptoms (10-50% pts with Wilson's disease)
Adverse effects requiring discontinuation of treatment
- Discontinued in 20-30% of pts with Wilson's disease
- Diarrhea (17%)
- Taste alteration (12%)
1-10%
Proteinuria (6%)
Rash (early and late 5%)
Thrombocytopenia (4-5%)
Leukopenia (2-5%)
Frequency not defined
Nausea/vomiting
Fever
Anorexia
Pemphigus
Oral lichenoid reaction
Myasthemia gravis
Neuropathy
Optic neuritisTinnitus
Goodpasture's syndrome
Renal failure
positive ANA
Hepatitis
Pancreatitis
Hemolytic anemia
Warnings
Contraindications
Penicillin allergy, discontinue if immune reactions
History of penicillamine-related aplastic anemia, agranulocytosis
Renal insufficiency (avoid if CrCl <50 mL/min)
Concurrency with gold salts, antimalarials, immunosuppressants, phenylbutazone
Cautions
Antacids, digoxin and iron (PO) decr levels and/or activity
Reactive airway disease
Associated with obliterative bronchiolitis
Increases the body's requirement for pyridoxine
Potential for development of (reversible) myasthenia gravis and other neurologic symptoms
Pregnancy and lactation
Pregnancy category: D; allowed only in Wilson's: not to exceed 750 mg/d; contraindicated for rheumatoid arthritis and cystinuria
Lactation: little information available, mfr states do not nurse
Pregnancy categories
A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.
B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.
C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.
D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.
X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.
NA: Information not available.
Pharmacology of Cuprimine, Depen (penicillamine)
Absorption: 40-70%
Peak Plasma Time: 1-3 hr
Peak Plasma Concentration: (250 mg dose) 1-2 mg/L
Metabolism: small amount in liver
Protein Bound: >80%
Excretion: urine
Mechanism of action
Chelates gold, copper, mercury, and arsenic
Cystinuria: forms disulfide bonds with cysteine and facilitates excretion of cysteine-penicillamine complex rather than cystine
Rheumatoid arthris: unknown; depresses T-cell activity in vitro
