Complications
Patients can present with acute or decompensated liver failure, including rapidly progressive ascites, hepatic encephalopathy, and bleeding diathesis.
Effective anti-copper therapy can improve neurologic symptoms.[5] However, paradoxical neurologic worsening can occur after initiation of chelation, or less commonly zinc therapy, usually in patients with neurologic symptoms at presentation.[5][51][52] Late neurologic worsening may also occur in patients who are asymptomatic or only have hepatic symptoms at presentation; this is usually related to treatment adherence.[52]
In one systematic review, 24.2% of patients with early neurologic worsening recovered completely, 27.3% recovered partially, and 39.8% did not improve (the remaining patients were lost to follow-up).[51]
In patients with neurologic symptoms, start chelation therapy slowly and closely monitor for paradoxical worsening during initiation. Guidelines recommend patients with neurologic symptoms have regular follow-up with a movement disorder specialist for at least 12 months after treatment initiation.[24]
Patients with Wilson disease with cirrhosis may have an increased risk of developing hepatocellular carcinoma, but the risk is lower compared with other chronic liver diseases.[67] In one retrospective review of three Dutch referral hospitals, the estimated annual risk of hepatocellular carcinoma was 0.09% in all patients with Wilson disease and 0.14% in patients with Wilson disease and cirrhosis.[68]
Screening and surveillance for hepatocellular carcinoma is recommended for patients with Wilson disease with cirrhosis.[5]
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