Hepatic and extrahepatic malignancies in autoimmune hepatitis. A long-term follow-up in 473 Swedish patients (original) (raw)
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Hepatocellular carcinoma in patients with autoimmune hepatitis
World Journal of Gastroenterology, 2009
AIM: To evaluate and confirm the low incidence of hepatocellular carcinoma (HCC) in patients with autoimmune hepatitis (AIH). At present only very few cases of HCC in patients with AIH and definite exclusion of chronic viral hepatitis have been published, suggesting that HCC due to AIH is rare. METHODS: In order to further investigate the incidence of HCC in patients with AIH, we reviewed our large cohort of 278 patients with AIH. RESULTS: Eighty-nine patients (32%) were diagnosed with liver cirrhosis, a preneoplastic condition for HCC. We studied a total of 431 patient years of cirrhosis in these patients, an average 4.8 years per patient. During this period none of the patients of our own study cohort developed HCC. However, three patients with HCC due to AIH associated liver cirrhosis were referred to our department for further treatment of HCC. In all three patients chronic viral hepatitis was excluded. CONCLUSION: We conclude that HCC may under rare circumstances develop due to chronic AIH dependent liver cirrhosis. Compared to other causes of liver cirrhosis such as chronic viral hepatitis, alcohol, or hemochromatosis, the incidence of HCC is significantly lower. Pathophysiological differences between AIH and chronic viral hepatitis responsible for differences in the incidence of HCC are yet to be further characterized and may lead to new therapeutic concepts in prevention and treatment of liver cancer.
Hepatocellular carcinoma in patients with autoimmune hepatitis: prevalence and risk factors
Journal of Hepatology, 2020
Background and aims. Liver cancer is one of the most common cause of deaths from cancer. Hepatocellular carcinoma (HCC) was reported at a frequency of 7% of patients with autoimmune hepatitis (AIH)-related cirrhosis in 1988. We aimed to provide a systematic literature review on the frequency of HCC in patients with AIH, after the discovery of hepatitis C virus (HCV), in order to avoid any possible confounding etiology. Methods. A literature search of the PubMed database between 1989-2016 was performed, using the relevant keywords "hepatocellular carcinoma" and "autoimmune hepatitis". We followed the PRISMA statement guidelines during the preparation of this review. Results. Eleven studies (n=8,460 patients with AIH) were retained for the final analysis. HCC was diagnosed in 0-12.3% of the AIH patients included in these studies. The overall occurrence of HCC in patients with AIH was estimated in two studies, at 5.1% and 6.2%, respectively. In patients with AIH and cirrhosis, the percentage of HCC varied between 0.2%-12.3%. The proportion of HCC in patients with AIH without cirrhosis was estimated at 1.03%. The percentage of cirrhosis in AIH patients varied from 18.7% to 83.3% in Japan, and from 12% to 50.2% in the other areas. The mean follow-up of the patients with AIH was of 10 years. Conclusions. The development of HCC in patients with AIH appeared to be similar before and after the discovery of HCV, and it was mainly associated to cirrhosis. The number of patients developing cirrhosis in relation with AIH was impressive. The long evolution of AIH to cirrhosis and, eventually, to HCC, has been be suggested.
The risk of liver cancer in autoimmune liver diseases
Therapeutic Advances in Medical Oncology
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 ...
Hepatology, 2008
Hepatocellular carcinoma (HCC) has traditionally been considered a rare complication of cirrhosis secondary to autoimmune hepatitis (AIH), yet the true incidence remains unknown due to a lack of published data. Consequently, some professional guidelines do not mandate routine surveillance for HCC in this condition. Our aims were to evaluate the rate at which HCC develops among a large, prospectively obtained cohort of patients with AIH at a single center. Demographic, clinical, and laboratory indices associated with the development of HCC were also identified. HCC was discovered in 15 of 243 patients with AIH, all of whom had type 1 AIH equating to 1090 cases per 100,000 patient follow-up years. HCC occurred in the same proportion of females as males, 6.1% versus 6.4%, P ؍ 0.95. HCC occurred more frequently in patients who had cirrhosis at presentation, 9.3% versus 3.4%, P ؍ 0.048, or who had a variceal bleed as the index presentation of AIH, 20% versus 5.3%, P ؍ 0.003. The median duration from time of confirmed cirrhosis to a diagnosis of HCC was 102.5 months, range 12-195 months. Median survival in patients whose HCC was diagnosed on surveillance was 19 months (range 6-36 months) compared with 2 months (range 0-14 months) for patients presenting symptomatically (P ؍ 0.042). Conclusion: Cirrhosis in AIH is the sine qua non for HCC development, which subsequently occurs at a rate of 1.1% per year and affects men and women in equal proportions. (HEPATOLOGY 2008;48:863-870.)
Hepatocellular carcinoma in autoimmune hepatitis
Digestive diseases and sciences, 2000
To determine if hepatocellular carcinoma can develop in autoimmune hepatitis in the absence of viral infection and to assess its frequency, liver tissue removed at hepatectomy was tested for HBV DNA and HCV RNA in one patient and the frequency of hepatocellular carcinoma was determined in 212 other uniformly followed individuals. The liver tissue from the propositus was uninfected and only one patient (0.5%) in the cohort undergoing routine follow-up developed malignancy during 1,732 patient-years of observation. Only one of 88 patients with cirrhosis (1%) developed hepatocellular carcinoma during 1,002 patient-years of observation after cirrhosis (mean, 123 +/- 9 months) and of the 65 patients with histological cirrhosis for at least five years, only one developed carcinoma during 162 +/- 8 months (incidence, 1 per 965 patient-years). We conclude that hepatocellular carcinoma can develop in autoimmune hepatitis in the absence of viral infection. Its occurrence is rare and only in l...
Autoimmune Hepatitis, Cirrhosis, and Hepatocellular Carcinoma (HCC
Digestive Diseases and Sciences, 2011
Hepatocellular carcinoma (HCC) is the fifth most common cancer worldwide and the third most common cause of cancer death. The success of treatment for HCC largely depends on the stage at which it is diagnosed, with 5-year survival rates of 70–75% in patients with compensated cirrhosis and early HCC [1–4]. As HCC usually arises in individuals with the clearly defined underlying risk factor of chronic liver disease, a focus on early detection through screening and surveillance programs in individuals with proven cirrhosis had been adopted by most clinicians [5, 6] despite an initial lack of supportive evidence. A randomised controlled trial of surveillance versus no surveillance performed in China in 2004 recruited nearly 19,000 patients having markers of current or past hepatitis B infection. Despite poor compliance, the HCC-related mortality in the surveillance arm was reduced by 37% [7]. It is not clear, however, whether screening is appropriate and cost effective for all aetiologies ...
World journal of gastroenterology : WJG, 2009
To characterize the clinical features of hepatocellular carcinoma (HCC) associated with autoimmune liver disease, we critically evaluated the literature on HCC associated with autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). A systematic review of the literature was conducted using the Japana Centra Revuo Medicina database which produced 38 cases of HCC with AIH (AIH-series) and 50 cases of HCC with PBC (PBC-series). We compared the clinical features of these two sets of patients with the general Japanese HCC population. On average, HCC was more common in men than in women with AIH or PBC. While many patients underwent chemolipiodolization (CL) or transcatheter arterial embolization (TAE) (AIH-series: P = 0.048 (vs operation), P = 0.018 (vs RFA, PEIT); PBC-series: P = 0.027 (vs RFA, PEIT), others refused therapeutic interventions [AIH-series: P = 0.038 (vs RFA, PEIT); PBC-series: P = 0.003 (vs RFA, PEIT)]. Liver failure was the primary cause of death among patients in...
Clinical features of hepatocellular carcinoma in patients with autoimmune hepatitis in Japan
Journal of Gastroenterology, 2013
Background The occurrence of hepatocellular carcinoma (HCC) in patients with autoimmune hepatitis (AIH) is rare compared to that in patients with viral hepatitis. To clarify the status of HCC in patients with AIH in Japan, the clinical features of HCC in patients with AIH were analyzed. Methods A primary survey gathered data from 496 member institutions of the Liver Cancer Study Group of Japan, and a secondary survey collected additional information from 250 HCC patients out of a total 4869 AIH patients who were identified in the primary survey. Results Of the 250 patients identified through the secondary survey, 127 patients (50.8 %) from throughout Japan were found to have HCC. The mean age at diagnosis of HCC was 69 years, and the male-to-female ratio was 1:5.7. The mean period from diagnosis of AIH to detection of HCC was 8 years, and Child-Pugh status at the time of HCC diagnosis was class A in 61.8 %; of the patients analyzed, 77.9 % also had cirrhosis of the liver. The mean value of maximum tumor diameter was 4.3 cm, and clinical stages were I in 20.1 % of patients, II in 47.6 %, III in 23.4 %, and IV in 8.9 %. The therapeutic modality used was surgery in 30.2 %, percutaneous therapy in 29.5 %, transcatheter arterial chemoembolization in 36.4 %. Cumulative survival rate was 85.4 % at one year. Conclusion The survey results showed that HCC developed in 5.1 % of patients with AIH in Japan, with cirrhosis of the liver commonly found in elderly individuals; when HCC was diagnosed at an early clinical stage, in many cases, the liver function was relatively preserved. After diagnosis of AIH, observation of its progression with close attention to potential HCC complications is necessary. Keywords Autoimmune hepatitis Á Hepatocellular carcinoma Á Cirrhosis Á Diabetes mellitus Á Liver Cancer Study Group of Japan Participating investigators other than the authors are listed in the Appendix.
Digestive Diseases and Sciences, 2019
Background: Hepatocellular carcinoma (HCC) is rare in patients with autoimmune hepatitis (AIH). However, the overall burden of AIH cirrhosis in causing HCC and patients' risk factors are not well understood. Aims: To characterize the proportion of HCC linked to AIH at a large academic health center, and to identify variables associated with HCC in patients with AIH in a case-control study design. Methods: Over a 14.5-year period, medical records of all patients with HCC were reviewed. Cases are AIH patients identified from the cohort and controls are patients with AIH without HCC. Three controls were randomly chosen from the Genetic Repository of Autoimmune Liver Disease and Coexisting Exposures database for each eligible case. Results: Out of 1,250 eligible patients, 20 were linked to AIH (1.6%). Their median age was 64 years, 40% men, and 100% Caucasian. 10% of AIH patients did not have evidence of cirrhosis at HCC diagnosis. The proportion of HCCs due to AIH decreased during the time intervals of the study. Compared to controls, cases were more likely men (40.0% vs. 18%, P=0.049), with longer AIH duration (median 16 years vs. 5 years, P=0.004). Prolonged AIH duration (OR 1.68, p=0.006) and older age (OR 1.15, p=0.049) were risk factors for HCC. Conclusions: AIH is a rare cause (1.6%) for HCC in Midwestern United States with a decreasing trend over 14.5 years. 10% of AIH-HCC patients occurs without cirrhosis. Patients with prolonged duration of the disease and older age are at high risk to develop HCC.