A clinical case of diagnosis of autoimmune hepatitis at the stage of liver cirrhosis
DOI:
https://doi.org/10.14739/2409-2932.2023.2.277495Keywords:
autoimmune hepatitis, cirrhosis, transaminases, autoantibodies, diagnosis, treatmentAbstract
The aim of the work is to analyze a clinical case of autoimmune hepatitis (АІН) diagnosis at the stage of cirrhosis in a 40-year-old female patient and to remind practicing doctors about the serious consequences of this disease.
Materials and methods. A clinical case of AIH diagnosed according to the criteria of the International Autoimmune Hepatitis Group (2008) at the stage of cirrhosis in a 40-year-old female patient was analyzed. A puncture liver biopsy with a morphological study of the hepatobioptate was also performed to verify the diagnosis.
Results. The analysis of the clinical case of AIH in a 40-year-old female patient revealed a delayed diagnosis of AIH at the cirrhosis stage. This was due to the absence of any clinical symptoms of the disease for a long period of time, and the lack of attention of practicing doctors in investigating the reasons for the long-term increase in transaminase activity. The trigger factor for the clinical manifestations of cirrhosis, which developed due to the long-term course of AIH, was emotional stress resulting from leaving the combat zone.
The application of the International Autoimmune Hepatitis Group criteria in clinical practice was demonstrated, specifically the combination of elevated antinuclear antibodies and total IgG, characteristic histological changes in the liver, and exclusion of hepatotropic viral infections, which allowed for a reliable diagnosis of AIH.
Medical treatment resulted in certain positive changes in clinical and laboratory indicators. However, according to the transplant doctor’s opinion, at this stage of the AIH course, the patient required a liver transplant and therefore was added to the waiting list.
Conclusions. This clinical case highlights the late diagnosis of AIH at the cirrhosis stage in a 40-year-old female patient due to the absence of clinical manifestations over several years and the lack of vigilance among doctors, possibly primary care physicians or specialists, to investigate the persistent increase in transaminase activity. The application of the International Autoimmune Hepatitis Group criteria helped confirm the diagnosis of AIH.
References
Hennes, E. M., Zeniya, M., Czaja, A. J., Parés, A., Dalekos, G. N., Krawitt, E. L., Bittencourt, P. L., Porta, G., Boberg, K. M., Hofer, H., Bianchi, F. B., Shibata, M., Schramm, C., Eisenmann de Torres, B., Galle, P. R., McFarlane, I., Dienes, H. P., Lohse, A. W., & International Autoimmune Hepatitis Group (2008). Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology (Baltimore, Md.), 48(1), 169-176. https://doi.org/10.1002/hep.22322
Ministry of Health of Ukraine. (2014, November 6). Pro zatverdzhennia ta vprovadzhennia medyko-tekhnolohichnykh dokumentiv zi standartyzatsii medychnoi dopomohy pry khronichnykh neinfektsiinykh hepatytakh. Nakaz MOZ Ukrainy vid 06.11.2014 r. No. 826 [On the approval and implementation of medical and technological documents on the standardization of medical care for chronic non-infectious hepatitis (No. 826)]. https://ips.ligazakon.net/document/MOZ24038
Liang, J. B., Chen, Y., Chen, R. L., Li, Y. K., Li, B., You, Z. R., Li, Y., Zhang, J., Huang, B. Y., Wei, Y. R., Lyu, Z. W., Lian, M., Xiao, X., Wang, Q. X., Tang, R. Q., Fang, J. Y., Chen, X. Y., Ma, X., & Miao, Q. (2021). CD8+ T cells actively penetrate hepatocytes via the CD44/p-ERM/F-actin pathway in autoimmune hepatitis. Journal of digestive diseases, 22(6), 351-362. https://doi.org/10.1111/1751-2980.12995
Heneghan, M. A., Yeoman, A. D., Verma, S., Smith, A. D., & Longhi, M. S. (2013). Autoimmune hepatitis. Lancet, 382(9902), 1433-1444. https://doi.org/10.1016/S0140-6736(12)62163-1
Tunio, N. A., Mansoor, E., Sheriff, M. Z., Cooper, G. S., Sclair, S. N., & Cohen, S. M. (2021). Epidemiology of Autoimmune Hepatitis (AIH) in the United States Between 2014 and 2019: A Population-based National Study. Journal of clinical gastroenterology, 55(10), 903-910. https://doi.org/10.1097/MCG.0000000000001449
European Association for the Study of the Liver (2015). EASL Clinical Practice Guidelines: Autoimmune hepatitis. Journal of hepatology, 63(4), 971-1004. https://doi.org/10.1016/j.jhep.2015.06.030
Trivedi, P. J., & Hirschfield, G. M. (2021). Recent advances in clinical practice: epidemiology of autoimmune liver diseases. Gut, 70(10), 1989-2003. https://doi.org/10.1136/gutjnl-2020-322362
Czaja, A. J. (2017). Global Disparities and Their Implications in the Occurrence and Outcome of Autoimmune Hepatitis. Digestive diseases and sciences, 62(9), 2277-2292. https://doi.org/10.1007/s10620-017-4675-y
Singal, A. K., Louvet, A., Shah, V. H., & Kamath, P. S. (2018). Grand Rounds: Alcoholic Hepatitis. Journal of hepatology, 69(2), 534-543. https://doi.org/10.1016/j.jhep.2018.05.001
Mack, C. L., Adams, D., Assis, D. N., Kerkar, N., Manns, M. P., Mayo, M. J., Vierling, J. M., Alsawas, M., Murad, M. H., & Czaja, A. J. (2020). Diagnosis and Management of Autoimmune Hepatitis in Adults and Children: 2019 Practice Guidance and Guidelines From the American Association for the Study of Liver Diseases. Hepatology (Baltimore, Md.), 72(2), 671-722. https://doi.org/10.1002/hep.31065
Floreani, A., Leung, P. S., & Gershwin, M. E. (2016). Environmental Basis of Autoimmunity. Clinical reviews in allergy & immunology, 50(3), 287-300. https://doi.org/10.1007/s12016-015-8493-8
Palle, S. K., Naik, K. B., McCracken, C. E., Kolachala, V. L., Romero, R., & Gupta, N. A. (2019). Racial disparities in presentation and outcomes of paediatric autoimmune hepatitis. Liver international, 39(5), 976-984. https://doi.org/10.1111/liv.14081
Ngu, J H., Bechly, K., Chapman, B. A., Burt, M. J., Barclay, M. L., Gearry, R. B., & Stedman, C. A. (2010). Population-based epidemiology study of autoimmune hepatitis: a disease of older women?. Journal of gastroenterology and hepatology, 25(10), 1681-1686. https://doi.org/10.1111/j.1440-1746.2010.06384.x
Kfuri, M., & Schatzker, J. (2019). Response to A. Kumar, et al., Letter to the Editor concerning "Revisiting the Schatzker classification of tibial plateau fractures" by Kfuri M, Schatzker J. Injury. 2018 49 December (12):2252-2263, Injury (2019) https://doi.org/10.1016/j.Injury.2019.01.020. Injury, 50(6), 1262-1264. https://doi.org/10.1016/j.injury.2019.04.010
Czaja, A. J. (2016). Diagnosis and Management of Autoimmune Hepatitis: Current Status and Future Directions. Gut and liver, 10(2), 177-203. https://doi.org/10.5009/gnl15352
Stravitz, R. T., Lefkowitch, J. H., Fontana, R. J., Gershwin, M. E., Leung, P. S., Sterling, R. K., Manns, M. P., Norman, G. L., Lee, W. M., & Acute Liver Failure Study Group (2011). Autoimmune acute liver failure: proposed clinical and histological criteria. Hepatology (Baltimore, Md.), 53(2), 517-526. https://doi.org/10.1002/hep.24080
Balitzer, D., Shafizadeh, N., Peters, M. G., Ferrell, L. D., Alshak, N., & Kakar, S. (2017). Autoimmune hepatitis: review of histologic features included in the simplified criteria proposed by the international autoimmune hepatitis group and proposal for new histologic criteria. Modern pathology, 30(5), 773-783. https://doi.org/10.1038/modpathol.2016.267
Terziroli Beretta-Piccoli, B., Mieli-Vergani, G., & Vergani, D. (2017). Autoimmune hepatitis: Standard treatment and systematic review of alternative treatments. World journal of gastroenterology, 23(33), 6030-6048. https://doi.org/10.3748/wjg.v23.i33.6030
Gatselis, N. K., Zachou, K., Koukoulis, G. K., & Dalekos, G. N. (2015). Autoimmune hepatitis, one disease with many faces: etiopathogenetic, clinico-laboratory and histological characteristics. World journal of gastroenterology, 21(1), 60-83. https://doi.org/10.3748/wjg.v21.i1.60
Muratori, P., Lalanne, C., Fabbri, A., Cassani, F., Lenzi, M., & Muratori, L. (2015). Type 1 and type 2 autoimmune hepatitis in adults share the same clinical phenotype. Alimentary pharmacology & therapeutics, 41(12), 1281-1287. https://doi.org/10.1111/apt.13210
Czaja, A. J. (2011). Performance parameters of the conventional serological markers for autoimmune hepatitis. Digestive diseases and sciences, 56(2), 545-554. https://doi.org/10.1007/s10620-010-1501-1
Efe, C., Ozaslan, E., Wahlin, S., Purnak, T., Muratori, L., Quarneti, C., Yüksel, O., & Muratori, P. (2013). Antibodies to soluble liver antigen in patients with various liver diseases: a multicentre study. Liver international, 33(2), 190-196. https://doi.org/10.1111/liv.12022
Czaja, A. J. (2010). Autoantibodies as prognostic markers in autoimmune liver disease. Digestive diseases and sciences, 55(8), 2144-2161. https://doi.org/10.1007/s10620-010-1268-4
Gregorio, G. V., McFarlane, B., Bracken, P., Vergani, D., & Mieli-Vergani, G. (2002). Organ and non-organ specific autoantibody titres and IgG levels as markers of disease activity: a longitudinal study in childhood autoimmune liver disease. Autoimmunity, 35(8), 515-519. https://doi.org/10.1080/0891693021000056721
Zachou, K., Oikonomou, K., Renaudineau, Y., Chauveau, A., Gatselis, N., Youinou, P., & Dalekos, G. N. (2012). Anti-α actinin antibodies as new predictors of response to treatment in autoimmune hepatitis type 1. Alimentary pharmacology & therapeutics, 35(1), 116-125. https://doi.org/10.1111/j.1365-2036.2011.04908.x
Mieli-Vergani, G., Vergani, D., Baumann, U., Czubkowski, P., Debray, D., Dezsofi, A., Fischler, B., Gupte, G., Hierro, L., Indolfi, G., Jahnel, J., Smets, F., Verkade, H. J., & Hadžić, N. (2018). Diagnosis and Management of Pediatric Autoimmune Liver Disease: ESPGHAN Hepatology Committee Position Statement. Journal of pediatric gastroenterology and nutrition, 66(2), 345-360. https://doi.org/10.1097/MPG.0000000000001801
Kirstein, M. M., Metzler, F., Geiger, E., Heinrich, E., Hallensleben, M., Manns, M. P., & Vogel, A. (2015). Prediction of short- and long-term outcome in patients with autoimmune hepatitis. Hepatology (Baltimore, Md.), 62(5), 1524-1535. https://doi.org/10.1002/hep.27983
Downloads
Published
How to Cite
Issue
Section
License
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access)