Floris F van den Brand1, Koen S van der Veen1, Ynto S de Boer1, Nicole M van Gerven1, Birgit I Lissenberg-Witte2, Ulrich Beuers3, Karel J van Erpecum4, Henk R van Buuren5, Jannie W den Ouden6, Johannus T Brouwer7, Jan M Vrolijk8, Robert C Verdonk9, Bart van Hoek10, Ger H Koek11, Joost P H Drenth12, Marleen M J Guichelaar13, Chris J J Mulder1, Elisabeth Bloemena14, Carin M J van Nieuwkerk1, Gerd Bouma15. 1. Amsterdam Gastroenterology and Metabolism, Department of Gastroenterology and Hepatology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, the Netherlands. 2. Department of Epidemiology and Biostatistics, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, the Netherlands. 3. Department of Gastroenterology and Hepatology, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands. 4. Department of Gastroenterology and Hepatology, University Medical Center, Utrecht, the Netherlands. 5. Department of Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, the Netherlands. 6. Department of Gastroenterology and Hepatology, Haga Hospital, The Hague, the Netherlands. 7. Department of Gastroenterology and Hepatology, Reinier de Graaf Groep, Delft, the Netherlands. 8. Department of Gastroenterology and Hepatology, Rijnstate Hospital, Arnhem, the Netherlands. 9. Department of Gastroenterology and Hepatology, Sint Antonius Hospital, Nieuwegein, the Netherlands. 10. Department of Gastroenterology and Hepatology, Leiden University Medical Center, Leiden, the Netherlands. 11. Department of Internal Medicine, division of Gastroenterology and Hepatology, Maastricht University Medical Center, Maastricht, the Netherlands. 12. Department of Gastroenterology and Hepatology, Radboud University Medical Center, Nijmegen, the Netherlands. 13. Department of Gastroenterology and Hepatology, Medisch Spectrum Twente, Enschede, the Netherlands. 14. Department of Pathology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, the Netherlands. 15. Amsterdam Gastroenterology and Metabolism, Department of Gastroenterology and Hepatology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, the Netherlands. Electronic address: g.bouma@vumc.nl.
Abstract
BACKGROUND & AIMS: There have been few reproducible studies of mortality in patients with autoimmune hepatitis (AIH) and its variants. We calculated mortality in a large national cohort of patients with AIH, with vs without cirrhosis, in the Netherlands. METHODS: We collected data from 449 patients with established AIH (77% female), from 6 academic and 10 non-academic hospitals in the Netherlands. We identified 29 patients with AIH and primary biliary cholangitis and 35 patients with AIH and primary sclerosing cholangitis (AIH-PSC). Mortality and liver transplantation data were assessed from August 1, 2006 through July 31, 2016. Standardized mortality ratios (SMR) were calculated using age-, sex-, and calendar year-matched mortality for the general Dutch population. RESULTS: During the 10-year follow-up period, 60 patients (13%) died (mean age, 71 years; range, 33-94 years). Twenty-six causes of death were liver related (43%), whereas the others could not be attributed to liver disease. Patients with AIH and cirrhosis had significantly higher mortality than the general population (SMR, 1.9; 95% CI, 1.2-3.4), whereas patients without cirrhosis did not (SMR, 1.2; 95% CI, 0.8-1.8). Patients with AIH-PSC had the largest increase in mortality, compared to the general population (SMR, 4.7; 95% CI, 1.5-14.6), of all groups analyzed. Mortality in patients with AIH and primary biliary cholangitis was not greater than the general population. Four or more relapses per decade or not achieving remission was associated with an increase in liver-related death or liver transplantation. Nine patients underwent liver transplantation; 2 died from non-liver related causes. Four of 9 patients on the waitlist for transplantation died before receiving a donated liver. CONCLUSION: In an analysis of data from a large national cohort of patients with AIH, we found increased mortality of patients with cirrhosis, but not of patients without cirrhosis, compared to the general Dutch population. Survival was significantly reduced in patients with AIH and features of concurrent PSC.
BACKGROUND & AIMS: There have been few reproducible studies of mortality in patients with autoimmune hepatitis (AIH) and its variants. We calculated mortality in a large national cohort of patients with AIH, with vs without cirrhosis, in the Netherlands. METHODS: We collected data from 449 patients with established AIH (77% female), from 6 academic and 10 non-academic hospitals in the Netherlands. We identified 29 patients with AIH and primary biliary cholangitis and 35 patients with AIH and primary sclerosing cholangitis (AIH-PSC). Mortality and liver transplantation data were assessed from August 1, 2006 through July 31, 2016. Standardized mortality ratios (SMR) were calculated using age-, sex-, and calendar year-matched mortality for the general Dutch population. RESULTS: During the 10-year follow-up period, 60 patients (13%) died (mean age, 71 years; range, 33-94 years). Twenty-six causes of death were liver related (43%), whereas the others could not be attributed to liver disease. Patients with AIH and cirrhosis had significantly higher mortality than the general population (SMR, 1.9; 95% CI, 1.2-3.4), whereas patients without cirrhosis did not (SMR, 1.2; 95% CI, 0.8-1.8). Patients with AIH-PSC had the largest increase in mortality, compared to the general population (SMR, 4.7; 95% CI, 1.5-14.6), of all groups analyzed. Mortality in patients with AIH and primary biliary cholangitis was not greater than the general population. Four or more relapses per decade or not achieving remission was associated with an increase in liver-related death or liver transplantation. Nine patients underwent liver transplantation; 2 died from non-liver related causes. Four of 9 patients on the waitlist for transplantation died before receiving a donated liver. CONCLUSION: In an analysis of data from a large national cohort of patients with AIH, we found increased mortality of patients with cirrhosis, but not of patients without cirrhosis, compared to the general Dutch population. Survival was significantly reduced in patients with AIH and features of concurrent PSC.
Authors: Floris F van den Brand; Koen S van der Veen; Birgit I Lissenberg-Witte; Ynto S de Boer; Bart van Hoek; Joost P H Drenth; Robert C Verdonk; Jan M Vrolijk; Carin M J van Nieuwkerk; Gerd Bouma Journal: Aliment Pharmacol Ther Date: 2019-10-15 Impact factor: 8.171
Authors: Maaike Biewenga; Akin Inderson; Maarten E Tushuizen; A Stijn L P Crobach; Bart van Hoek Journal: Liver Transpl Date: 2020-10-27 Impact factor: 5.799
Authors: Maaike Biewenga; Xavier P D M J Verhelst; Martine A M C Baven-Pronk; Hein Putter; Aad P van den Berg; Karin C M J van Nieuwkerk; Henk R van Buuren; Gerd Bouma; Ynte S de Boer; Cedric Simoen; Isabelle Colle; Jeoffrey Schouten; Filip Sermon; Christophe van Steenkiste; Hans van Vlierberghe; Adriaan J van der Meer; Frederik Nevens; Bart van Hoek Journal: United European Gastroenterol J Date: 2021-06-24 Impact factor: 4.623