Autoimmune liver diseases. Clinical and laboratory findings in patients in a national reference hospital
DOI:
https://doi.org/10.52784/27112330.140Keywords:
autoimmunity, transaminases, autoantibodies, autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, overlap syndrome.Abstract
Introduction. Autoimmune liver diseases are a group of pathologies characterized by an autoimmune response against hepatocytes and/or the biliary epithelium. Their clinical manifestations are varied, with alterations in liver function tests and the presence of autoantibodies. Methodology. Descriptive study with 101 patients who attended at the Hospital Universitario de La Samaritana in Bogota D.C., between January and December 2019, with the diagnoses of autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis and overlap syndrome. Clinical and laboratory parameters were evaluated in order to characterize their frequency in these pathologies, due to the importance of an early diagnosis. Results. There were 54 cases of autoimmune hepatitis,
19 cases of primary biliary cholangitis, 4 cases of primary sclerosing cholangitis, and 24 cases of overlap syndrome. Of all patients, 81% were women, the average age was 55 years, and 39% had cirrhosis. In general, the findings were consistent with what has been described worldwide, such as a higher prevalence in women and autoimmune comorbidity. Conclusion. The findings indicate that any alteration in the liver biochemical profile should be considered to rule out an autoimmune liver disease for an early diagnosis, avoiding the possibility of cirrhosis and its complications, with the need for a liver transplant as the only therapeutic alternative.
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References
Manns MP, Czaja AJ, Gorham JD, Krawitt EL, Mieli-Vergani G, Vergani D, et al. Diagnosis and management of autoimmune hepatitis. Hepatology 2010;51:2193-2213. https://doi.org/10.1002/hep.23584.
Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ. Primary biliary cirrhosis. Hepatology 2009;50:291-308. https://doi.org/10.1002/hep.22906.
Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology 2010;51:660-678. https://doi.org/10.1002/hep.23294.
Donaldson PT. Genetics of liver disease: immunogenetics and disease pathogenesis. Gut 2004;53:599-608. https://doi.org/10.1136/gut.2003.031732.
Trivedi PJ, Hirschfield GM. Review article: overlap syndromes and autoimmune liver disease. Aliment Pharmacol Ther 2012;36:517-533. https://doi.org/10.1111/j.1365-2036.2012.05223.x.
Robinson MW, Harmon C, O'Farrelly C. Liver immunology and its role in inflammation and homeostasis. Cell Mol Immunol 2016;13:267-276. https://doi.org/10.1038/cmi.2016.3.
Orts-Costa JA, Zúñiga-Cabrera A, Alarcón-Torres I. Hepatitis autoinmune. An Med Interna 2004;21:34-48.
Mieli-Vergani G, Vergani D, Czaja AJ, Manns MP, Krawitt EL, Vierling JM, et al. Autoimmune hepatitis. Nat Rev Dis Primers 2018;4:18017. https://doi.org/10.1038/nrdp.2018.17.
Carbone M, Neuberger JM. Autoimmune liver disease, autoimmunity and liver transplantation. J Hepatol 2014;60:210-223. https://doi.org/10.1016/j.jhep.2013.09.020.
Floreani A, Leung PS, Gershwin ME. Environmental basis of autoimmunity. Clin Rev Allergy Immunol 2016;50:287-300. https://doi.org/10.1007/s12016-015-8493-8.
Czaja AJ. Examining pathogenic concepts of autoimmune hepatitis for cues to future investigations and interventions. World J Gastroenterol 2019;25:6579-6606. https://doi.org/10.3748/wjg.v25.i45.6579.
Flores A, Mayo MJ. Primary biliary cirrhosis in 2014. Curr Opin Gastroenterol 2014;30:245-252. https://doi.org/10.1097/mog.0000000000000058.
Sener AG. Autoantibodies in autoimmune liver diseases. Apmis 2015;123:915-919. https://doi.org/10.1111/apm.12442.
Horsley-Silva JL, Carey EJ, Lindor KD. Advances in primary sclerosing cholangitis. Lancet Gastroenterol Hepatol 2016;1:68-77. https://doi.org/10.1016/s2468-1253(16)30010-3.
Orellana N I, Valera M JM, Nei L C, Poniachik T J, Berger F Z, Latorre M R, et al. Colangitis esclerosante primaria: revisión de 12 años en dos centros de referencia. Rev Med Chile 2005;133:776-780. https://doi.org/10.4067/S0034-98872005000700004.
Williamson KD, Chapman RW. Primary sclerosing cholangitis: a clinical update. Br Med Bull 2015;114:53-64. https://doi.org/10.1093/bmb/ldv019.
Poblet G, López-Manzanares J. Hepatitis Autoinmune. Protocolos diagnóstico-terapéuticos de Gastroenterología, Hepatología y Nutrición Pediátrica SEGHNP-AEP. Madrid: Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátrica; 2010. p. 211-220.
Chazouillères O, Wendum D, Serfaty L, Montembault S, Rosmorduc O, Poupon R. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology 1998;28:296-301. https://doi.org/10.1002/hep.510280203.
Chapman MH, Thorburn D, Hirschfield GM, Webster GGJ, Rushbrook SM, Alexander G, et al. British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis. Gut 2019;68:1356-1378. https://doi.org/10.1136/gutjnl-2018-317993.
Aguilar-Nájera O, Velasco-Zamora JA, Torre A. Diagnóstico y tratamiento de los síndromes de sobreposición de hepatitis autoinmune. Rev Gastroenterol Mex 2015;80:150-159. https://doi.org/10.1016/j.rgmx.2015.04.001.
Hennes EM, Zeniya M, Czaja AJ, Parés A, Dalekos GN, Krawitt EL, et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology 2008;48:169-176. https://doi.org/10.1002/hep.22322.
Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary biliary cholangitis: 2018 practice guidance from the American Association for the Study of Liver Diseases. Hepatology 2019;69:394-419. https://doi.org/10.1002/hep.30145.
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;8:530-534. https://doi.org/10.1016/j.cgh.2010.03.004.
García-Torres ML, Primo J, Ortuño JA, Martínez M, Antón MD, Zaragoza A, et al. Estudio clínico de la hepatitis autoinmune del adulto en Valencia. Rev Esp Enferm Dig 2008;100:400-404.
Rodríguez RH, Samada SM, Hernández PJC ea. Caracterización de pacientes con enfermedades hepáticas autoinmunes. Invest Medicoquir 2014;6:214-228.
Pape S, Gevers TJG, Vrolijk JM, van Hoek B, Bouma G, van Nieuwkerk CMJ, et al. Rapid response to treatment of autoimmune hepatitis associated with remission at 6 and 12 months. Clin Gastroenterol Hepatol 2020;18:1609-1617.e1604. https://doi.org/10.1016/j.cgh.2019.11.013.
Fickert P, Hirschfield GM, Denk G, Marschall HU, Altorjay I, Färkkilä M, et al. norUrsodeoxycholic acid improves cholestasis in primary sclerosing cholangitis. J Hepatol 2017;67:549-558. https://doi.org/10.1016/j.jhep.2017.05.009.
Liang H, Manne S, Shick J, Lissoos T, Dolin P. Incidence, prevalence, and natural history of primary sclerosing cholangitis in the United Kingdom. Medicine (Baltimore) 2017;96:e7116. https://doi.org/10.1097/md.0000000000007116.
Rupp C, Rössler A, Zhou T, Rauber C, Friedrich K, Wannhoff A, et al. Impact of age at diagnosis on disease progression in patients with primary sclerosing cholangitis. United European Gastroenterol J 2018;6:255-262. https://doi.org/10.1177/2050640617717156.
Sebode M, Weiler-Normann C, Liwinski T, Schramm C. Autoantibodies in autoimmune liver disease-clinical and diagnostic relevance. Front Immunol 2018;9:609. https://doi.org/10.3389/fimmu.2018.00609.
Brahim I, Brahim I, Hazime R, Admou B. [Autoimmune hepatitis: Immunological diagnosis]. Presse Med 2017;46:1008-1019. https://doi.org/10.1016/j.lpm.2017.08.012.
Sahebjam F, Vierling JM. Autoimmune hepatitis. Front Med 2015;9:187-219. https://doi.org/10.1007/s11684-015-0386-y.
Liberal R, Krawitt EL, Vierling JM, Manns MP, Mieli-Vergani G, Vergani D. Cutting edge issues in autoimmune hepatitis. J Autoimmun 2016;75:6-19. https://doi.org/10.1016/j.jaut.2016.07.005.
Thiele DL. Autoimmune hepatitis. Clin Liver Dis 2005;9:635-646. https://doi.org/10.1016/j.cld.2005.07.004.
Díaz-Ramírez GS, Martínez-Casas OY, Marín-Zuluaga JI, Donado-Gómez JH, Muñoz-Maya O, Santos-Sánchez O, et al. Características diferenciales de la hepatitis autoinmune en adultos mayores colombianos: estudio de cohorte. Rev Colomb Gastroenterol 2019;2:135-143. https://doi.org/10.22516/25007440.291.
Mahmud N, Doshi SD, Forde KA, Khungar V. Transient elastography reliably estimates liver fibrosis in autoimmune hepatitis. Clin Exp Hepatol 2019;5:244-249. https://doi.org/10.5114/ceh.2019.87639.
van den Brand FF, van der Veen KS, Lissenberg-Witte BI, de Boer YS, van Hoek B, Drenth JPH, et al. Adverse events related to low dose corticosteroids in autoimmune hepatitis. Aliment Pharmacol Ther 2019;50:1120-1126. https://doi.org/10.1111/apt.15528.
Czaja AJ, Souto EO, Bittencourt PL, Cancado EL, Porta G, Goldberg AC, et al. Clinical distinctions and pathogenic implications of type 1 autoimmune hepatitis in Brazil and the United States. J Hepatol 2002;37:302-308. https://doi.org/10.1016/s0168-8278(02)00182-4.
Czaja AJ. Acute and acute severe (fulminant) autoimmune hepatitis. Dig Dis Sci 2013;58:897-914. https://doi.org/10.1007/s10620-012-2445-4.
Bittencourt PL, Farias AQ, Porta G, Cançado EL, Miura I, Pugliese R, et al. Frequency of concurrent autoimmune disorders in patients with autoimmune hepatitis: effect of age, gender, and genetic background. J Clin Gastroenterol 2008;42:300-305. https://doi.org/10.1097/MCG.0b013e31802dbdfc.
Valera M JM, Smok S G, Poniachik T J, Oksenberg R D, Silva P G, Ferrario B M, et al. Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia. Rev Med Chile 2006;134:469-474. https://doi.org/10.4067/S0034-98872006000400010.
Sánchez A, Hernández N, Chiodi D, Berrueta J, Robaina G, Pollio C, et al. Cirrosis biliar primaria: Aspectos clínico- epidemiológicos en una población uruguaya. Acta Gastroenterol Latinoam 2013;43:288-293.
Zandanell S, Strasser M, Feldman A, Tevini J, Strebinger G, Niederseer D, et al. Low rate of new-onset primary biliary cholangitis in a cohort of anti-mitochondrial antibody-positive subjects over six years of follow-up. J Intern Med 2020;287:395-404. https://doi.org/10.1111/joim.13005.
Vespasiani-Gentilucci U, Rosina F, Pace-Palitti V, Sacco R, Pellicelli A, Chessa L, et al. Rate of non-response to ursodeoxycholic acid in a large real-world cohort of primary biliary cholangitis patients in Italy. Scand J Gastroenterol 2019;54:1274-1282. https://doi.org/10.1080/00365521.2019.1669702.
Murillo-Perez C. Prognostic factors for clinical outcomes in patients with primary biliary cholangitis. Países bajos: Erasmus University Rotterdam; 2021. Jan 8. Acceso 12 de abril de 2021. Disponible en http://hdl.handle.net/1765/134765.
González-Huezo MS, Delgado-Ayala LY, Osorio-Núñez AL, Meléndez-Mercado C. Asociaciones autoinmunes en una cohorte mexicana con colangitis biliar primaria. Rev Gastroenterol Mex 2019;84:130-135. https://doi.org/10.1016/j.rgmx.2018.03.008.
Tsuneyama K, Baba H, Morimoto Y, Tsunematsu T, Ogawa H. Primary biliary cholangitis: Its pathological characteristics and immunopathological mechanisms. J Med Invest 2017;64:7-13. https://doi.org/10.2152/jmi.64.7.
Murillo-Perez CF, Hirschfield GM, Corpechot C, Floreani A, Mayo MJ, van der Meer A, et al. Fibrosis stage is an independent predictor of outcome in primary biliary cholangitis despite biochemical treatment response. Aliment Pharmacol Ther 2019;50:1127-1136. https://doi.org/10.1111/apt.15533.
Fülöp T, Dupuis G, Witkowski JM, Larbi A. The role of immunosenescence in the development of age-related diseases. Rev Invest Clin 2016;68:84-91.
Kumagai J, Taida T, Ogasawara S, Nakagawa T, Iino Y, Shingyoji A, et al. Clinical characteristics and outcomes of primary sclerosing cholangitis and ulcerative colitis in Japanese patients. PLoS One 2018;13:e0209352. https://doi.org/10.1371/journal.pone.0209352.
Park Y, Cho Y, Cho EJ, Kim YJ. Retrospective analysis of autoimmune hepatitis-primary biliary cirrhosis overlap syndrome in Korea: characteristics, treatments, and outcomes. Clin Mol Hepatol 2015;21:150-157. https://doi.org/10.3350/cmh.2015.21.2.150.
Zhao P, Han Y. Low incidence of positive smooth muscle antibody and high incidence of isolated IgM elevation in Chinese patients with autoimmune hepatitis and primary biliary cirrhosis overlap syndrome: a retrospective study. BMC Gastroenterol 2012;12:1. https://doi.org/10.1186/1471-230x-12-1.
Nguyen HH, Shaheen AA, Baeza N, Lytvyak E, Urbanski SJ, Mason AL, et al. Evaluation of classical and novel autoantibodies for the diagnosis of primary biliary cholangitis-autoimmune hepatitis overlap syndrome (PBC-AIH OS). PLoS One 2018;13:e0193960. https://doi.org/10.1371/journal.pone.0193960.
Wu H-M, Sheng L, Wang Q, Bao H, Miao Q, Xiao X, et al. Performance of transient elastography in assessing liver fibrosis in patients with autoimmune hepatitis-primary biliary cholangitis overlap syndrome. World J Gastroenterol 2018;24:737-743. https://doi.org/10.3748/wjg.v24.i6.737.
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