TY - JOUR
T1 - No increased risk of hepatocellular carcinoma in cirrhosis due to Wilson disease during long-term follow-up
AU - van Meer, Suzanne
AU - de Man, Robert A.
AU - van den Berg, Aad P.
AU - Houwen, Roderick H. J.
AU - Linn, Francisca H. H.
AU - van Oijen, Martijn G. H.
AU - Siersema, PD
AU - van Erpecum, Karel J.
PY - 2015/3
Y1 - 2015/3
N2 - Background and AimsData on risk of hepatocellular carcinoma (HCC) in patients with Wilson disease are scarce. We determine HCC risk in a well-defined cohort of Wilson patients.MethodsAll patients with a confirmed diagnosis of Wilson disease (Leipzig score4) in three Dutch university referral hospitals were included in this retrospective cohort study. End of follow-up was defined as date of diagnosis of HCC, liver transplantation, death, or last available hospital visit. Also, a meta-analysis was performed to determine incidence and mortality rate of HCC in Wilson disease based on all published cohorts.ResultsIn total, 130 patients with Wilson disease were followed during a median follow-up of 15 years (range 0.1-51.2). At baseline, cirrhosis was present in 74 patients (57% of total: 64% compensated, and 36% decompensated). At end of follow-up, liver disease severity was improved, stable or deteriorated in 20%, 46%, and 24% of all cases (10% unknown), respectively. Two patients developed HCC (one despite excellent decoppering after 50 years follow-up, the other with newly diagnosed Wilson disease). Estimated annual HCC risk for all patients was 0.09% (95% confidence interval [CI]: 0.01-0.28). Subgroup analysis in cirrhotic patients revealed an annual HCC risk of 0.14% (95% CI: 0.02-0.46). The meta-analysis showed an annual HCC risk of 0.04% (95% CI: 0.01-0.10) and HCC mortality rate of 2.6/10000 person-years (95% CI: 0.7-7.0).ConclusionsEven in case of cirrhosis, HCC risk is low in Wilson disease. Our data do not support regular HCC surveillance in Wilson disease.
AB - Background and AimsData on risk of hepatocellular carcinoma (HCC) in patients with Wilson disease are scarce. We determine HCC risk in a well-defined cohort of Wilson patients.MethodsAll patients with a confirmed diagnosis of Wilson disease (Leipzig score4) in three Dutch university referral hospitals were included in this retrospective cohort study. End of follow-up was defined as date of diagnosis of HCC, liver transplantation, death, or last available hospital visit. Also, a meta-analysis was performed to determine incidence and mortality rate of HCC in Wilson disease based on all published cohorts.ResultsIn total, 130 patients with Wilson disease were followed during a median follow-up of 15 years (range 0.1-51.2). At baseline, cirrhosis was present in 74 patients (57% of total: 64% compensated, and 36% decompensated). At end of follow-up, liver disease severity was improved, stable or deteriorated in 20%, 46%, and 24% of all cases (10% unknown), respectively. Two patients developed HCC (one despite excellent decoppering after 50 years follow-up, the other with newly diagnosed Wilson disease). Estimated annual HCC risk for all patients was 0.09% (95% confidence interval [CI]: 0.01-0.28). Subgroup analysis in cirrhotic patients revealed an annual HCC risk of 0.14% (95% CI: 0.02-0.46). The meta-analysis showed an annual HCC risk of 0.04% (95% CI: 0.01-0.10) and HCC mortality rate of 2.6/10000 person-years (95% CI: 0.7-7.0).ConclusionsEven in case of cirrhosis, HCC risk is low in Wilson disease. Our data do not support regular HCC surveillance in Wilson disease.
KW - cirrhosis
KW - hepatocellular carcinoma
KW - Wilson disease
KW - CLINICAL PRESENTATION
KW - COPPER
KW - CARCINOGENESIS
KW - COHORT
KW - MUTATIONS
KW - DIAGNOSIS
UR - http://www.ncbi.nlm.nih.gov/pubmed/25160780
U2 - 10.1111/jgh.12716
DO - 10.1111/jgh.12716
M3 - Article
SN - 0815-9319
VL - 30
SP - 535
EP - 539
JO - Journal of Gastroenterology and Hepatology
JF - Journal of Gastroenterology and Hepatology
IS - 3
ER -