penicillamine

Action and Spectrum

Actions and Spectrum: 

• penicillamine is a medication used primarily to treat Wilson’s disease, rheumatoid arthritis, and other autoimmune disorders. Its mechanism of action involves its ability to bind with and remove excess copper from the body, which is beneficial in treating Wilson’s disease. 

• It also has immunomodulatory properties that effectively treat autoimmune disorders like rheumatoid arthritis. It is believed to work by interfering with the formation of disulphide bonds, which are necessary for the proper function of specific immune cells. 
• penicillamine has a broad spectrum of activity against various autoimmune disorders, including rheumatoid arthritis, lupus, and scleroderma. It is also used to treat cystinuria, a genetic disorder that causes the formation of cystine stones in the urinary tract 

Drug Interaction

Adverse Reaction

Frequency defined 

>10% 

Worsening neurologic symptoms 

Adverse effects indicating therapy discontinuation 

Taste alteration (12%) 

Discontinued in 20-30% of pts with Wilson’s disease 

Diarrhea (17%) 

1-10% 

Rash(<5%) 

Leukopenia(2-5%) 

Proteinuria (6%) 

Thrombocytopenia (4-5%) 

Frequency not defined 

Goodpasture’s syndrome 

Neuropathy 

Oral lichenoid reaction 

Optic neuritisTinnitus 

Positive ANA 

Hepatitis 

Pancreatitis 

Hemolytic anemia 

Anorexia 

Myasthenia gravis 

Nausea/vomiting 

Fever 

Black Box Warning

Contraindication / Caution

Contraindications/caution: 

Contraindications: 

• Hypersensitivity or allergy to penicillamine: If a person has a history of allergic reactions to penicillamine or other medications, they should not take it. 
• Severe kidney disease: penicillamine can worsen kidney function in people with severe kidney disease, so it is contraindicated in this population 
• Lupus erythematosus: penicillamine can trigger or worsen symptoms of lupus erythematosus, a chronic autoimmune disease, which is contraindicated in people with this condition. 
• Blood disorders: penicillamine can cause blood disorders, so it is contraindicated in people with a history of these conditions. 

• Parkinson’s disease: penicillamine can worsen symptoms of Parkinson’s disease, so it is contraindicated in people with this condition 

Caution: 

• Renal impairment: It can cause kidney damage or worsen pre-existing kidney disease. Monitoring kidney functions regularly and adjusting the dosage as needed is essential. 

• Hepatic impairment: It can cause liver damage or worsen pre-existing liver disease. Monitoring liver functions regularly and adjusting the dosage as needed is essential. 

• Hematologic abnormalities: It can cause a decrease in white blood cells, red blood cells, and platelets. Regular blood tests should be done to monitor for these abnormalities. 

• Skin reactions can cause skin rashes, including severe allergic reactions like Stevens-Johnson syndrome. If a rash or other skin reaction occurs, the medication should be discontinued and medical attention sought. 

• Vitamin B6 deficiency: It can cause a deficiency in vitamin B6, which can lead to nerve damage. Vitamin B6 supplements may be recommended while taking penicillamine. 

• Drug interactions: It can interact with other medications, such as non-steroidal anti-inflammatory drugs (NSAIDs) and gold compounds, and may require dosage adjustments or discontinuation of one of the medications. 

Pregnancy / Lactation

Pregnancy consideration: penicillamine is not recommended during pregnancy due to the potential risks to the developing fetus or infant. 

Lactation: Excretion of the drug in human breast milk is unknown 

Pregnancy category: 

Category A: well-controlled and Satisfactory studies show no risk to the fetus in the first or later trimester.   

Category B: there was no evidence of risk to the fetus in animal studies, and there were not enough studies on pregnant women. 

Category C: there was evidence of risk of adverse effects in animal reproduction studies, and no adequate evidence in human studies must take care of potential risks in pregnant women.    

Category D: adequate data with sufficient evidence of human fetal risk from various platforms, but despite the potential risk, and used only in emergency cases for potential benefits.    

Category X: Drugs listed in this category outweigh the risks over benefits. Hence these categories of drugs need to be avoided by pregnant women.    

Category N: There is no data available for the drug under this category 

Pharmacology

Pharmacology: 

• penicillamine acts by binding with copper in the bloodstream and then transporting the copper to the liver, where it is eliminated from the body through bile. 
• penicillamine also has immunomodulatory properties, which can help regulate the immune system. It works by disrupting the formation of disulfide bonds, which are essential for the structure and function of specific immune cells. This can be helpful in conditions such as rheumatoid arthritis, where an overactive immune system contributes to joint inflammation. 

Pharmacodynamics: 

• The pharmacodynamics of penicillamine involves its ability to bind with and remove excess copper from the body and its immunomodulatory effects on the immune system. 
• Copper is an essential trace element that is critical in several physiological processes. However, in certain medical conditions such as Wilson’s disease, copper can accumulate in the body to toxic levels. penicillamine works by forming stable complexes with copper in the bloodstream and promoting its excretion from the body through the liver and bile. 
• In addition to its chelating effects, penicillamine has immunomodulatory properties that make it effective in treating autoimmune disorders such as rheumatoid arthritis. It is believed to work by interfering with the formation of disulfide bonds, which are necessary for the proper function of specific immune cells. This can help to reduce inflammation and joint damage in conditions such as rheumatoid arthritis. 

Pharmacokinetics: 

Absorption 

penicillamine is rapidly absorbed from the gastrointestinal tract when taken orally, with peak plasma concentrations achieved within 1 to 2 hours after administration. 

Distribution 

penicillamine is distributed widely throughout the body, including the liver, kidney, and brain. The medication can cross the blood-brain barrier and the placenta and is excreted into breast milk. 

Metabolism 

penicillamine is not metabolized to a significant extent in the body. 

Elimination and Excretion 

penicillamine is primarily excreted unchanged in the urine. The medication has a short half-life of approximately 1 to 2 hours and is rapidly eliminated from the body. 

Adminstartion

Administration: 

penicillamine is typically administered orally in the form of a tablet or capsule. It is usually taken on an empty stomach one hour before or two hours after meals to maximize its absorption 

Patient Information Leaflet

Patient information leaflet 

Generic Name: penicillamine 

Pronounced: [ pen-ih-SILL-ah-meen ] 

Why do we use penicillamine? 

penicillamine is sometimes used for the treatment of mercury poisoning. when mercury enters the body, it binds to sulfhydryl groups, which are present in many proteins and enzymes. penicillamine contains a sulfhydryl group that can compete with the mercury for binding sites, allowing it to bind to the mercury instead. Once penicillamine has bound to the mercury, it can form a stable complex that is more easily excreted by the kidneys.

Penicillamine is not always the first-line treatment for mercury poisoning, and its use depends on the severity of the poisoning, as well as the patient’s overall health and medical history.

penicillamine is primarily used for the treatment of a few specific conditions, including: 

• Wilson’s disease: It is used to treat Wilson’s disease, a genetic disorder that causes copper to accumulate in the body, leading to liver and brain damage. Penicillamine works by binding to copper in the body, allowing it to be excreted in the urine. 

• Rheumatoid arthritis: It is used as a disease-modifying antirheumatic drug (DMARD) in treating rheumatoid arthritis. The medication can slow the progression of joint damage and reduce inflammation. 

• Cystinuria: It is sometimes used to treat cystinuria, a rare genetic disorder that causes the buildup of cystine in the urine, leading to kidney stones. 

• Pemphigus: It may be used as a treatment for pemphigus, a rare autoimmune disorder that causes blistering of the skin and mucous membranes. 

• Scleroderma: It has been used to treat scleroderma, a connective tissue disorder that causes the thickening and hardening of the skin and other tissues