Treatments for Wilson disease

DrugMode of actionNeurologic deteriorationSide effects
D-penicillamineChelator, induces cupruria10%–20% during initial phase of treatmentFever, rash, lupus reaction, leukopenia, aplastic anemia, nephrotic syndrome, hepatotoxicity, retinitis
TrientineChelator, induces cupruria10%–15% during initial phase of treatmentGastritis
Aplastic anemia
TetrathiomolybdateAims to reduce level of free serum copperDeveloped to protect central nervous system and other tissues vulnerable to free copperAnemia
ZincBlocks intestinal absorption of copperNoGastritis
  • Based on information in Roberts EA, Schilsky ML; American Association for Study of Liver Diseases (AASLD). Diagnosis and treatment of Wilson disease: an update. Hepatology 2008; 47:2089–2111.