Wilson disease maintenance therapy

Autosomal recessive disorder of copper excretion leading to copper accumulation in the liver, brain, and cornea. Zinc acetate is used as maintenance therapy after the initial chelation phase (D-penicillamine, trientine), blocking intestinal copper absorption.

ICD-10 E83.01 drug

Also known as: Hepatolenticular degeneration

Drugs used for this condition

AZinc (oral salts: picolinate, citrate, gluconate, oxide, acetate)First line · Mineral supplements. Trace elementsZinc acetate (Wilzin, Galzin) or zinc sulphate is maintenance therapy for Wilson disease after the initial copper chelation phase. Dose: 50 mg elemental zinc three times daily. Included in EASL 2012 and AASLD 2023 guidelines as maintenance therapy. In asymptomatic ATP7B mutation carriers, it is first-line therapy. The effect develops over 4–8 weeks and requires lifelong use.

How to read tiers A–F

Evidence tiers belong to the drug-indication pair, not to the drug as a whole: the same substance can be A for one condition and F for another.

AStrong evidence – first or second line in international guidelines
BRecommended in selected cases
CStudy data contradict each other
DBenefit not proven, no documented harm
FDocumented harm, or risk without benefit