A clinical case of diagnosis of autoimmune hepatitis at the stage of liver cirrhosis

Authors

DOI:

https://doi.org/10.14739/2409-2932.2023.2.277495

Keywords:

autoimmune hepatitis, cirrhosis, transaminases, autoantibodies, diagnosis, treatment

Abstract

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.

Author Biographies

O. A. Svitlytska, Educational and Scientific Medical Center “University Clinic” of Zaporizhzhia State Medical and Pharmaceutical University, Ukraine

MD, PhD, Head of the Multimodal Pathology Department

O. V. Riabokon, Zaporizhzhia State Medical and Pharmaceutical University, Ukraine

MD, PhD, DSc, Professor, Head of the Department of Infectious Diseases

A. O. Svitlytskyі, Zaporizhzhia State Medical and Pharmaceutical University, Ukraine

MD, PhD, Associate Professor of the Department of Human Anatomy, Operative Surgery and Topographic Anatomy

N. H. Horbatkova, Educational and Scientific Medical Center “University Clinic” of Zaporizhzhia State Medical and Pharmaceutical University, Ukraine

Medical Director

E. V. Kotliarevska, Educational and Scientific Medical Center “University Clinic” of Zaporizhzhia State Medical and Pharmaceutical University, Ukraine

Director

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

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Published

2023-07-03

How to Cite

1.
Svitlytska OA, Riabokon OV, Svitlytskyі AO, Horbatkova NH, Kotliarevska EV. A clinical case of diagnosis of autoimmune hepatitis at the stage of liver cirrhosis. Current issues in pharmacy and medicine: science and practice [Internet]. 2023Jul.3 [cited 2024Oct.30];16(2):194-200. Available from: http://pharmed.zsmu.edu.ua/article/view/277495

Issue

Vol. 16 No. 2 (2023)

Section

Case Reports

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