TY - JOUR
T1 - The risk of liver cancer in autoimmune liver diseases
AU - Lleo, Ana
AU - de Boer, Ynto S.
AU - Liberal, Rodrigo
AU - Colombo, Massimo
N1 - Publisher Copyright: © The Author(s), 2019. Copyright: Copyright 2019 Elsevier B.V., All rights reserved.
PY - 2019/7/1
Y1 - 2019/7/1
N2 - Hepatocellular carcinoma (HCC), the dominant primary malignancy of the liver, has almost invariably a fatal outcome that can be averted only by early diagnosis and treatment. While the close association of HCC with chronic viral hepatitis and alcohol abuse has impacted favourably on screening and treatment of this deadly tumour, at the same time it has long obscured the etiologic role of autoimmune liver diseases. Recently, a systematic analysis of 25 published cohorts disclosed a 3.1 × 1000 patients/year incidence of HCC in autoimmune hepatitis patients that tripled in those with cirrhosis. HCC is also a sequela of primary biliary cholangitis, where the incidence is more relevant in males, those with advanced liver disease and nonresponders to ursodeoxycholic acid therapy. Cholangiocarcinoma (CCA), the second ranking primary cancer of the liver, is also on the rise with its intrahepatic pattern, in part reflecting an association with chronic liver diseases of diverse aetiology. In the USA and northern Europe, perihilar CCA is a frequent complication of primary sclerosing cholangitis, a cholestatic disorder thought to be immune mediated. International Guidelines clearly recommend HCC screening with abdominal ultrasonography every 6 months in autoimmune cirrhotic patients. While surveillance of patients with autoimmune liver disorders who are at risk of HCC affects both early diagnosis and radical therapy of this tumour, this is not the case for CCA, where early diagnosis is challenged by the lack of sensitive and accurate tests for screening.
AB - Hepatocellular carcinoma (HCC), the dominant primary malignancy of the liver, has almost invariably a fatal outcome that can be averted only by early diagnosis and treatment. While the close association of HCC with chronic viral hepatitis and alcohol abuse has impacted favourably on screening and treatment of this deadly tumour, at the same time it has long obscured the etiologic role of autoimmune liver diseases. Recently, a systematic analysis of 25 published cohorts disclosed a 3.1 × 1000 patients/year incidence of HCC in autoimmune hepatitis patients that tripled in those with cirrhosis. HCC is also a sequela of primary biliary cholangitis, where the incidence is more relevant in males, those with advanced liver disease and nonresponders to ursodeoxycholic acid therapy. Cholangiocarcinoma (CCA), the second ranking primary cancer of the liver, is also on the rise with its intrahepatic pattern, in part reflecting an association with chronic liver diseases of diverse aetiology. In the USA and northern Europe, perihilar CCA is a frequent complication of primary sclerosing cholangitis, a cholestatic disorder thought to be immune mediated. International Guidelines clearly recommend HCC screening with abdominal ultrasonography every 6 months in autoimmune cirrhotic patients. While surveillance of patients with autoimmune liver disorders who are at risk of HCC affects both early diagnosis and radical therapy of this tumour, this is not the case for CCA, where early diagnosis is challenged by the lack of sensitive and accurate tests for screening.
KW - autoimmune hepatitis
KW - autoimmune liver diseases
KW - cholangiocarcinoma
KW - hepatic cancer
KW - hepatocellular carcinoma
KW - primary biliary cholangitis
KW - primary sclerosing cholangitis
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85068964180&origin=inward
UR - https://www.ncbi.nlm.nih.gov/pubmed/31320937
UR - http://www.scopus.com/inward/record.url?scp=85068964180&partnerID=8YFLogxK
U2 - https://doi.org/10.1177/1758835919861914
DO - https://doi.org/10.1177/1758835919861914
M3 - Review article
C2 - 31320937
SN - 1758-8340
VL - 11
JO - Therapeutic advances in medical oncology
JF - Therapeutic advances in medical oncology
ER -