Uses

Penicillamine is indicated for the treatment of Wilson's disease (to remove excess copper), cystinuria (to reduce urinary cystine and prevent stone formation), and severe active rheumatoid arthritis unresponsive to conventional therapy. It is not effective for ankylosing spondylitis.

Do not use if

Contraindicated in pregnancy (except for Wilson's disease or cystinuria), in patients with a history of penicillamine-induced aplastic anemia or agranulocytosis, in those with renal insufficiency (except Wilson's disease), and during breastfeeding.

Side effects

Penicillamine has a high incidence of adverse reactions, including proteinuria, nephrotic syndrome, hematological toxicity (thrombocytopenia, leukopenia, aplastic anemia), rash, taste disturbance, and autoimmune syndromes such as myasthenia gravis and lupus-like syndrome.

Warnings

Requires close and continuous medical supervision throughout treatment. Monitor blood counts, urinalysis, and renal function regularly. Use with extreme caution in penicillin-allergic patients. Concurrent use of gold, antimalarials, or immunosuppressive drugs with penicillamine is generally not recommended.

How it works

Penicillamine is a chelating agent that combines with copper, mercury, lead, and other heavy metals to form stable complexes excreted in the urine. In cystinuria, it reacts with cystine to form soluble mixed disulfides. Its mechanism in rheumatoid arthritis is not fully elucidated but may involve immune modulation.

Storage

Store at controlled room temperature, protected from moisture and heat. Keep in a tightly closed container.