Treatment of Wilson's Disease in Decompensated Cirrhosis Without Hepatic Encephalopathy
This protocol addresses Wilson's disease presenting with decompensated cirrhosis in the absence of hepatic encephalopathy. Patients in this setting may respond to medical therapy — though response typically requires more than three months — and concurrent evaluation for liver transplantation (LT) is indicated alongside medical management from the outset.
The regimen centres on chelation therapy combined with concurrent evaluation for liver transplantation. An intensive combination approach is also described within this protocol — the specific agents involved, how they are sequenced, and the clinical conditions guiding their use are detailed in full in the structured regimen.
The primary target is improvement of prothrombin time, with initial response expected no earlier than one month after starting treatment, and normalisation anticipated over a period of three months to one year.
References
DOI: 10.1016/j.jhep.2024.11.007
Patients with decompensated cirrhosis in WD may respond to medical therapy, usually after >3 months of treatment, but they should be concomitantly evaluated for LT (LoE 3, strong recommendation, strong consensus).
Recently, “combination” therapy with either D-penicillamine or trientine plus zinc has been proposed as an intensive regimen for patients with decompensated liver disease.
Response to medical treatment may take time, with improvement of prothrombin time after a minimum of 1 month and normalisation between 3 months and 1 year or more.
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