Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med. 2005;353:1261–73.

CAS  PubMed  Google Scholar 

Lleo A, Wang G-Q, Gershwin ME, et al. Primary biliary cholangitis. Lancet. 2020;396:1915–26.

CAS  PubMed  Google Scholar 

Trivella J, John BV, Levy C. Primary biliary cholangitis: Epidemiology, prognosis, and treatment. Hepatol Commun. 2023;7: e0179.

PubMed  PubMed Central  Google Scholar 

Umemura T, Joshita S, Saito H, et al. KIR/HLA genotypes confer susceptibility and progression in patients with autoimmune hepatitis. JHEP Reports. 2019;1:353–60.

PubMed  PubMed Central  Google Scholar 

Mieli-Vergani G, Vergani D, Czaja AJ, et al. Autoimmune hepatitis. Nat Rev Dis Primers. 2018;4:1–21.

Google Scholar 

Terziroli Beretta-Piccoli B, Mieli-Vergani G, Vergani D. Chapter 44—Autoimmune hepatitis. In: Gershwin ME, Tsokos GC, Diamond B, editors. The Rose and Mackay textbook of autoimmune diseases. 7th ed. Academic Press; 2024. p. 869–904.

Gerussi A, Invernizzi P, Carbone M. Challenges in the diagnosis and management of AIH-PBC syndrome. JHEP Rep. 2025;7:101224.

PubMed  Google Scholar 

Bajaj JS, O’Leary JG, Lai JC, et al. Acute-on-chronic liver failure clinical guidelines. Official J Am Coll Gastroenterol ACG. 2022;117:225–52.

Google Scholar 

Nakayama N, Uemura H, Uchida Y, et al. A multicenter pilot survey to clarify the clinical features of patients with acute-on-chronic liver failure in Japan. Hepatol Res. 2018;48:303–12.

PubMed  Google Scholar 

Tanaka A, Harada K. Acute presentation of autoimmune hepatitis–from acute hepatitis to ALF and ACLF. Hepatol Int. 2024;18:1385–95.

PubMed  Google Scholar 

Iwadare T, Kimura T, Yamashita Y, et al. Serum autotaxin levels predict liver-related events in patients with primary biliary cholangitis: a long-term multicenter observational study. Clin Transl Gastroenterol. 2024. https://doi.org/10.14309/ctg.0000000000000779.

Article  PubMed  PubMed Central  Google Scholar 

Uojima H, Yamasaki K, Sugiyama M, et al. Quantitative measurements of M2BPGi depend on liver fibrosis and inflammation. J Gastroenterol. 2024. https://doi.org/10.1007/s00535-024-02100-3.

Article  PubMed  Google Scholar 

Sarin SK, Choudhury A, Sharma MK, et al. Acute-on-chronic liver failure: consensus recommendations of the Asian Pacific association for the study of the liver (APASL): an update. Hepatol Int. 2019;13:353–90.

PubMed  Google Scholar 

Moreau R, Jalan R, Gines P, et al. Acute-on-chronic liver failure is a distinct syndrome that develops in patients with acute decompensation of cirrhosis. Gastroenterology. 2013;144(1426–37): e9.

Google Scholar 

Jalan R, Saliba F, Pavesi M, et al. Development and validation of a prognostic score to predict mortality in patients with acute-on-chronic liver failure. J Hepatol. 2014;61:1038–47.

PubMed  Google Scholar 

Bajaj JS, O’Leary JG, Reddy KR, et al. Survival in infection-related acute-on-chronic liver failure is defined by extrahepatic organ failures. Hepatology. 2014;60:250–6.

PubMed  Google Scholar 

Danan G, Teschke R. RUCAM in drug and herb induced liver injury: the update. Int J Mol Sci. 2015;17:14.

PubMed  PubMed Central  Google Scholar 

Danan G, Benichou C. Causality assessment of adverse reactions to drugs—I. a novel method based on the conclusions of international consensus meetings: application to drug-induced liver injuries. J Clin Epidemiol. 1993;46:1323–30.

CAS  PubMed  Google Scholar 

Moreau R, Gao B, Papp M, et al. Acute-on-chronic liver failure: a distinct clinical syndrome. J Hepatol. 2021;75:S27–35.

PubMed  Google Scholar 

Anand L, Choudhury A, Bihari C, et al. Flare of autoimmune hepatitis causing acute on chronic liver failure: diagnosis and response to corticosteroid therapy. Hepatology. 2019;70:587–96.

CAS  PubMed  Google Scholar 

Gupta T, Dhiman RK, Rathi S, et al. Impact of hepatic and extrahepatic insults on the outcome of acute-on-chronic liver failure. J Clin Exp Hepatol. 2017;7:9–15.

PubMed  Google Scholar 

Antunes AG, Teixeira C, Vaz AM, et al. Comparison of the prognostic value of chronic liver failure consortium scores and traditional models for predicting mortality in patients with cirrhosis. Gastroenterol Hepatol. 2017;40:276–85 (English Edition).

PubMed  Google Scholar 

Shen ZX, Wu DD, Xia J, et al. Prevalence and clinical characteristics of autoimmune liver disease in hospitalized patients with cirrhosis and acute decompensation in China. World J Gastroenterol. 2022;28(31):4417–30. https://doi.org/10.3748/wjg.v28.i31.4417.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Nakayama N, Uemura H, Uchida Y, et al. Nationwide survey for patients with acute-on-chronic liver failure occurring between 2017 and 2019 and diagnosed according to proposed Japanese criteria. J Gastroenterol. 2021;56:1092–106. https://doi.org/10.1007/s00535-021-01834-8.

Article  PubMed  Google Scholar 

Thanapirom K, Teerasarntipan T, Treeprasertsuk S, et al. Impact of compensated cirrhosis on survival in patients with acute-on-chronic liver failure. Hepatol Int. 2022;16:171–82. https://doi.org/10.1007/s12072-021-10266-8.

Article  PubMed  Google Scholar 

Benítez C, Arnold J, Cambindo V, et al. Effect of acute on chronic liver failure over post-transplant survival. Ann Hepatol. 2023;28: 101128. https://doi.org/10.1016/j.aohep.2023.101128.

Article  CAS  PubMed  Google Scholar 

Singal AK, Wong RJ, Jalan R, et al. Primary biliary cholangitis has the highest waitlist mortality in patients with cirrhosis and acute on chronic liver failure awaiting liver transplant. Clin Transplant. 2021;35: e14479. https://doi.org/10.1111/ctr.14479.

Article  PubMed  Google Scholar 

Philips CA, Schnabl B, Bajaj JS. Gut microbiome and alcohol-associated liver disease. J Clin Exp Hepatol. 2022;12:1349–59. https://doi.org/10.1016/j.jceh.2021.12.016.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Chen VC, Joseph CR, Chan WOY, et al. Inflammasome-driven fatal acute-on-chronic liver failure triggered by mild COVID-19. Viruses. 2024. https://doi.org/10.3390/v16101646.

Article  PubMed  PubMed Central  Google Scholar 

Premkumar M, Anand AC. Tobacco, cigarettes, and the liver: the smoking gun. J Clin Exp Hepatol. 2021;11:700–12. https://doi.org/10.1016/j.jceh.2021.07.016.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Taneja S, Mehtani R, De A, et al. Spectrum of autoimmune liver disease and real-world treatment experience from a tertiary care hospital. J Clin Exp Hepatol. 2023;13:241–51. https://doi.org/10.1016/j.jceh.2022.11.002.

Article  CAS  PubMed  Google Scholar 

Goel R, Eapen CE. Recognizing dysfunctional innate and adaptive immune responses contributing to liver damage in patients with cirrhosis. J Clin Exp Hepatol. 2022;12:993–1002. https://doi.org/10.1016/j.jceh.2021.10.001.

Article  CAS  PubMed  Google Scholar 

Rashidi-Alavijeh J, Nuruzade N, Frey A, et al. Implications of anaemia and response to anaemia treatment on outcomes in patients with cirrhosis. JHEP Rep. 2023;5: 100688. https://doi.org/10.1016/j.jhepr.2023.100688.

Article  PubMed  PubMed Central  Google Scholar 

Lu Q, Yu J, Xia N, et al. Obeticholic acid aggravates liver fibrosis by activating hepatic farnesoid X receptor-induced apoptosis in cholestatic mice. Chem Biol Interact. 2025;406: 111364. https://doi.org/10.1016/j.cbi.2024.111364.

Article  CAS  PubMed  Google Scholar 

Kuiper EM, Zondervan PE, Van Buuren HR. Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome. Clin Gastroenterol Hepatol. 2010;8:530–4.

PubMed  Google Scholar 

Nakanuma Y, Zen Y, Harada K, et al. Application of a new histological staging and grading system for primary biliary cirrhosis to liver biopsy specimens: interobserver agreement. Pathol Int. 2010;60:167–74.

PubMed  Google Scholar 

Nikias GA, Batts KP, Czaja AJ. The nature and prognostic implications of autoimmune hepatitis with an acute presentation. J Hepatol. 1994;21:866–71.

CAS  PubMed  Google Scholar 

Okano N, Yamamoto K, Sakaguchi K, et al. Clinicopathological features of acute-onset autoimmune hepatitis. Hepatol Res. 2003;25:263–70.

PubMed