Аннотация
Первичный билиарный холангит (ПБХ) – хроническое аутоиммунное холестатическое заболевание печени, характеризующееся опосредованным Т-лимфоцитами повреждением внутрипеченочных билиарных эпителиальных клеток и наличием специфического серологического маркера – антимитохондриальных антител, при отсутствии лечения приводящее к терминальной стадии билиарного цирроза печени. ПБХ остается серьезной проблемой общественного здравоохранения. До недавнего времени ПБХ обозначали как «первичный билиарный цирроз», однако заболевание часто диагностируется в доцирротическую фазу, когда еще нет сформированного цирроза, а есть лишь угроза его развития. Смена дефиниции болезни гармонично укладывается в рамки концепции превентивной медицины, поскольку своевременной постановкой диагноза и адекватной терапией эту фазу заболевания можно продлить на годы и десятилетия, однако все эти годы пациента «травмирует» заранее поставленный диагноз цирроза печени. В конце предшествующего десятилетия было опубликовано большое число международных клинических рекомендаций и гайдлайнов, посвященных ПБХ. В рамках представленного обзора о современном состоянии проблемы ПБХ использованы публикации из различных международных баз данных с акцентом на публикации текущего десятилетия, в которых отмечается важность ранней диагностики заболевания с акцентом на биохимические маркеры холестаза (прежде всего щелочную фосфатазу) и иммунологические маркеры (в первую очередь антимитохондриальные антитела) и раннего начала лечения с применением препарата 1-й линии – урсодезоксихолевой кислоты.
Ключевые слова: первичный билиарный холангит, дефиниция болезни, эпидемиология, диагностика, щелочная фосфатаза, антимитохондриальные антитела, клиника, лечение, урсодезоксихолевая кислота, обетихолевая кислота, мониторинг, прогноз.
Об авторе
Д.И. Трухан
ФГБОУ ВО «Омский государственный медицинский университет» Минздрава России, Омск, Россия
dmitry_trukhan@mail.ru
Список литературы
1. Zhao Y, Yin Z, Du H et al. The latest research trends in primary biliary cholangitis: a bibliometric analysis. Clin Exp Med 2022 Apr 7. DOI: 10.1007/s10238-022-00825-0
2. Xu H, Yanny B. Primary Biliary Cholangitis: A Review. Gene Expression 2022; 21 (2): 45–50. DOI: 10.14218/GEJLR.2022.00013
3. Tanaka A, Hirohara J, Nakano T et al. Association of bezafibrate with transplant-free survival in patients with primary biliary cholangitis. J Hepatol 2021; 75 (3): 565–71. DOI: 10.1016/j.jhep.2021.04.010
4. Трухан Д.И., Викторова И.А., Сафонов А.Д. Болезни печени. СПб.: Фолиант, 2010. URL: https://www.elibrary.ru/item.asp? id=19551702 [Truhan D.I., Viktorova I.A., Safonov A.D. Bolezni pecheni. Saint Petersburg: Foliant, 2010. URL: https://www.elibrary.ru/item.asp? id=19551702 (in Russian).]
5. Beuers U, Gershwin ME, Gish RG et al. Changing nomenclature for PBC: from ‘cirrhosis’ to ‘cholangitis’. J Hepatol 2015; 63: 1285–7. DOI: 10.1016/j.jhep.2015.06.031
6. Трухан Д.И., Викторова И.А., Сафонов А.Д. Болезни печени. СПб.: СпецЛит, 2019. URL: https://www.elibrary.ru/item.asp? id=41392560 [Truhan D.I., Viktorova I.A., Safonov A.D. Bolezni pecheni. Saint Petersburg: SpecLit, 2019. URL: https://www.elibrary.ru/item.asp? id=41392560 (in Russian).]
7. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. J Hepatol 2017; 67 (1): 145–72. DOI: 10.1016/j.jhep.2017.03.022
8. Hirschfield GM, Dyson JK, Alexander GJM et al. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut 2018; 67 (9): 1568–94. DOI: 10.1136/gutjnl-2017-315259
9. Younossi ZM, Bernstein D, Shiffman ML et al. Diagnosis and Management of Primary Biliary Cholangitis. Am J Gastroenterol 2019; 114 (1): 48–63. DOI: 10.1038/s41395-018-0390-3
10. Lindor KD, Bowlus CL, Boyer J et al. Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases. Hepatology 2019; 69 (1): 394–19. DOI: 10.1002/hep.30145
11. Leung KK, Deeb M, Hirschfield GM. Review article: pathophysiology and management of primary biliary cholangitis. Aliment Pharmacol Ther 2020; 52 (7): 1150–64. DOI: 10.1111/apt.16023
12. Chinese Society of Hepatology, Chinese Medical Association. Guidelines on the diagnosis and management of primary biliary cholangitis (2021). Zhonghua Nei Ke Za Zhi 2021; 60 (12): 1024–37. DOI: 10.3760/cma.j.cn112138-20211112-00794
13. Smith A, Giles B, Aspinall RJ. Primary biliary cholangitis: advances in understanding and management. Br J Hosp Med (Lond) 20222; 83 (3): 1–9. DOI: 10.12968/hmed.2021.0450
14. Imam MH, Lindor KD. The natural history of primary biliary cirrhosis. Semin Liver Dis 2014; 34 (3): 329–33. DOI: 10.1055/s-0034-1383731
15. Lu M, Li J, Haller IV et al. Factors Associated with Prevalence and Treatment of Primary Biliary Cholangitis in United States Health Systems. Clin Gastroenterol Hepatol 2018; 16 (8): 1333–41.e6. DOI: 10.1016/j.cgh.2017.10.018
16. Wibawa IDN, Shalim CP. Geographical Disparity in Primary Biliary Cholangitis Prevalence: A Mini-review. Gene Expression 2022; 21 (2): 41–44. DOI: 10.14218/GE.2022.00005
17. Gulamhusein AF, Hirschfield GM. Primary biliary cholangitis: pathogenesis and therapeutic opportunities. Nat Rev Gastroenterol Hepatol 2020; 17 (2): 93–110. DOI: 10.1038/s41575-019-0226-7
18. Al-Harthy N, Kumagi T. Natural history and management of primary biliary cirrhosis. Hepat Med 2012; 4: 61–71. DOI: 10.2147/HMER. S25998
19. Lazaridis KN, Talwalkar JA. Clinical epidemiology of primary biliary cirrhosis: incidence, prevalence, and impact of therapy. J Clin Gastroenterol 2007; 41 (5): 494–500. DOI: 10.1097/01.mcg. 0000225653.07932.8f
20. Tanaka A. Current understanding of primary biliary cholangitis. Clin Mol Hepatol 2021; 27 (1): 1–21. DOI: 10.3350/cmh.2020.0028
21. Lleo A, Jepsen P, Morenghi E et al. Evolving Trends in Female to Male Incidence and Male Mortality of Primary Biliary Cholangitis. Sci Rep 2016; 6: 25906. DOI: 10.1038/srep25906
22. Smyk DS, Rigopoulou EI, Pares A et al. Sex differences associated with primary biliary cirrhosis. Clin Dev Immunol 2012; 2012: 610504. DOI: 10.1155/2012/610504
23. Carbone M, Mells GF, Pells G et al. Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic acid. Gastroenterology 2013; 144 (3): 560–9. e7; quiz e13-4. DOI: 10.1053/j.gastro.2012.12.005
24. Kim KA, Ki M, Choi HY et al. Population-based epidemiology of primary biliary cirrhosis in South Korea. Aliment Pharmacol Ther 2016; 43 (1): 154–62. DOI: 10.1111/apt.13448
25. Invernizzi F, Cilla M, Trapani S et al. Gender and Autoimmune Liver Diseases: Relevant Aspects in Clinical Practice. J Pers Med 2022; 12 (6): 925. DOI: 10.3390/jpm12060925
26. Jeong SH. Current epidemiology and clinical characteristics of autoimmune liver diseases in South Korea. Clin Mol Hepatol 2018; 24 (1): 10–9. DOI: 10.3350/cmh.2017.0066
27. Gatselis NK, Zachou K, Lygoura V et al. Geoepidemiology, clinical manifestations and outcome of primary biliary cholangitis in Greece. Eur J Intern Med 2017; 42: 81–8. DOI: 10.1016/j.ejim.2017.05.006
28. Kanth R, Shrestha RB, Rai I et al. Incidence of Primary Biliary Cholangitis in a Rural Midwestern Population. Clin Med Res 2017; 15 (1–2): 13–8. DOI: 10.3121/cmr.2017.1351
29. James OF, Bhopal R, Howel D et al. Primary biliary cirrhosis once rare, now common in the United Kingdom? Hepatology 1999; 30 (2): 390–4. DOI: 10.1002/hep.510300213
30. Sood S, Gow PJ, Christie JM, Angus PW. Epidemiology of primary biliary cirrhosis in Victoria, Australia: high prevalence in migrant populations. Gastroenterology 2004; 127 (2): 470–5. DOI: 10.1053/j.gastro.2004.04.064
31. Myers RP, Shaheen AA, Fong A et al. Epidemiology and natural history of primary biliary cirrhosis in a Canadian health region: a population-based study. Hepatology 2009; 50 (6): 1884–92. DOI: 10.1002/hep.23210
32. Lu M, Zhou Y, Haller IV et al. Increasing Prevalence of Primary Biliary Cholangitis and Reduced Mortality With Treatment. Clin Gastroenterol Hepatol 2018; 16 (8): 1342–1350.e1.
33. Tanaka A, Mori M, Matsumoto K et al. Increase trend in the prevalence and male-to-female ratio of primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis in Japan. Hepatol Res 2019; 49: 881–9. DOI: 10.1111/hepr.13342
34. Li H, Guan Y, Han C et al. The pathogenesis, models and therapeutic advances of primary biliary cholangitis. Biomed Pharmacother 2021; 140: 111754. DOI: 10.1016/j.biopha.2021.111754
35. Liu X, Invernizzi P, Lu Y et al. Genome-wide meta-analyses identify three loci associated with primary biliary cirrhosis. Nat Genet 2010; 42 (8): 658–60. DOI: 10.1038/ng.627
36. Hirschfield GM, Heathcote EJ, Gershwin ME. Pathogenesis of cholestatic liver disease and therapeutic approaches. Gastroenterology 2010; 139 (5): 1481–96. DOI: 10.1053/j.gastro.2010.09.004
37. Selmi C, Invernizzi P, Zuin M et al. Genetics and geoepidemiology of primary biliary cirrhosis: Following the footprints to disease etiology. Semin Liver Dis 2005; 25: 265–80. DOI: 10.1055/s-2005-916319
38. Selmi C, Mayo MJ, Bach N et al. Primary biliary cirrhosis in monozygotic and dizygotic twins: genetics, epigenetics, and environment. Gastroenterology 2004; 127 (2): 485–92. DOI: 10.1053/j.gastro.2004.05.005
39. Örnolfsson KT, Olafsson S, Bergmann OM et al. Using the Icelandic genealogical database to define the familial risk of primary biliary cholangitis. Hepatology 2018; 68 (1): 166–71. DOI: 10.1002/hep.29675
40. Gulamhusein AF, Juran BD, Lazaridis KN. GWAS in Primary Biliary Cirrhosis. Semin Liver Dis 2015; 35: 392 DOI: 10.1055/s-0035- 1567831
41. Tanaka A, Leung PS, Gershwin ME. Environmental basis of primary biliary cholangitis. Exp Biol Med (Maywood) 2018; 243 (2): 184–9. DOI: 10.1177/1535370217748893
42. Burroughs AK, Rosenstein IJ, Epstein O et al. Bacteriuria and primary biliary cirrhosis. Gut 1984; 25 (2): 133–7. DOI: 10.1136/gut.25.2.133
43. Wang JJ, Yang GX, Zhang WC et al. Escherichia coli infection induces autoimmune cholangitis and anti-mitochondrial antibodies in nonobese diabetic (NOD). B6 (Idd10/Idd18) mice. Clin Exp Immunol 2014; 175 (2): 192–201. DOI: 10.1111/cei.12224
44. Shimoda S, Nakamura M, Ishibashi H et al. HLA DRB4 0101-restricted immunodominant T cell autoepitope of pyruvate dehydrogenase Литература / References | КЛИНИЧЕСКИЙ РАЗБОР В ОБщЕЙ МЕДИЦИНЕ | ТОМ 4 | №2 | 2023 | Clinical review for general practice | vol. 4 | no. 2 | 2023 | 57 Обзор / Review complex in primary biliary cirrhosis: evidence of molecular mimicry in human autoimmune diseases. J Exp Med 1995; 181 (5): 1835–45. DOI: 10.1084/jem.181.5.1835
45. Tanaka A, Leung PSC, Gershwin ME. Pathogen infections and primary biliary cholangitis. Clin Exp Immunol 2018; 195: 25–34. DOI: 10.1111/cei.13198
46. Juran BD, Lazaridis KN. Environmental factors in primary biliary cirrhosis. Semin Liver Dis 2014; 34: 265–72. DOI: 10.1055/s-0034- 1383726
47. Amano K, Leung PS, Rieger R et al. Chemical xenobiotics and mitochondrial autoantigens in primary biliary cirrhosis: Identification of antibodies against a common environmental, cosmetic, and food additive, 2-octynoic acid. J Immunol 2005; 174 (9): 5874–83.
48. Rieger R, Leung PS, Jeddeloh MR et al. Identification of 2-nonynoic acid, a cosmetic component, as a potential trigger of primary biliary cirrhosis. J Autoimmun 2006; 27 (1): 7–16.
49. Gershwin ME, Selmi C, Worman HJ et al. Risk factors and comorbidities in primary biliary cirrhosis: A controlled interview-based study of 1032 patients. Hepatology 2005; 42: 1194–202. DOI: 10.1002/hep.20907
50. Tanaka A. PBC: No Longer a Western Disease? Clin Liver Dis 2020; 16: 227–30. DOI: 10.1002/cld.903
51. Acharya C, Sahingur SE, Bajaj JS. Microbiota, cirrhosis, and the emerging oral-gut-liver axis. JCI Insight 2017; 2 (19): e94416. DOI: 10.1172/jci.insight.94416
52. Fukui H. Increased Intestinal Permeability and Decreased Barrier Function: Does It Really Influence the Risk of Inflammation? Inflamm Intest Dis 2016; 1 (3): 135–45. DOI: 10.1159/000447252
53. Симаненков В.И., Маев И.В., Ткачева О.Н. и др. Синдром повышенной эпителиальной проницаемости в клинической практике. Мультидисциплинарный национальный консенсус. Кардиоваскулярная терапия и профилактика. 2021; 20 (1): 2758. DOI: 10.15829/1728-8800-2021-2758 [Simanenkov V.I., Maev I.V., Tkacheva O.N. et al. Syndrome of increased epithelial permeability in clinical practice. Multidisciplinary national Consensus. Cardiovascular Therapy and Prevention. 2021; 20 (1): 2758. DOI: 10.15829/1728-8800-2021-2758 (in Russian).]
54. Симаненков В.И., Маев И.В., Ткачева О.Н. и др. Эпителий-протективная терапия при коморбидных заболеваниях. Практические рекомендации для врачей. Терапевтический архив. 2022; 94 (8): 6–22. DOI: 10.26442/00403660.2022.08.20152 [Simanenkov V.I., Maev I.V., Tkacheva O.N. et al. Epithelial protective therapy in comorbid diseases. Practical Guidelines for Physicians. Terapevticheskii Arkhiv. 2022; 94 (8): 6–22. DOI: 10.26442/00403660.2022.08.20152 (in Russian).]
55. Ampuero J, Lucena A, Hernández-Guerra M et al. Primary biliary cholangitis and SARS-CoV-2 infection: incidence, susceptibility and outcomes. Gut 2021; Dec 7: gutjnl-2021-325700. DOI: 10.1136/ gutjnl-2021-325700
56. Прашнова М.К., Райхельсон К.Л., Марченко Н.В., Захаренко С.М. СOVID-19 у пациентов с первичным билиарным холангитом. Российский журнал гастроэнтерологии, гепатологии, колопроктологии. 2022; 32 (3): 29–34. DOI: 10.22416/1382-4376-2022-32-3-29-34 [Prashnova M.K., Rajhel'son K.L., Marchenko N.V., Zaharenko S.M. SOVID-19 u pacientov s pervichnym biliarnym holangitom. Rossijskij zhurnal gastroenterologii, gepatologii, koloproktologii. 2022; 32 (3): 29–34. DOI: 10.22416/1382-4376-2022-32-3-29-34 (in Russian).]
57. Brevini T, Maes M, Webb GJ et al. FXR inhibition may protect from SARS-CoV-2 infection by reducing ACE2. Nature 2022; Dec 5. DOI: 10.1038/s41586-022-05594-0
58. Lammers WJ, van Buuren HR, Hirschfield GM et al. Levels of alkaline phosphatase and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis: an international follow-up study. Gastroenterology 2014; 147 (6): 1338–49. e5; quiz e15. DOI: 10.1053/j.gastro.2014.08.029
59. Purohit T, Cappell MS. Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy. World J Hepatol 2015; 7 (7): 926–41. DOI: 10.4254/wjh.v7.i7.926
60. Трухан Д.И., Филимонов С.Н. Дифференциальный диагноз основных гастроэнтерологических синдромов и симптомов. М.: Практическая медицина, 2016. URL: https://elibrary.ru/ item.asp?id=27499786 [Truhan D.I., Filimonov S.N. Differencial'nyj diagnoz osnovnyh gastroenterologicheskih sindromov i simptomov. Moscow: Prakticheskaya medicina, 2016. URL: https://elibrary.ru/item.asp?id=27499786 (in Russian).]
61. Трухан Д.И., Филимонов С.Н. Дифференциальный диагноз основных симптомов и синдромов при заболеваниях желудочно-кишечного тракта. Новокузнецк: Полиграфист, 2022. URL: https: //elibrary.ru/item.asp?id=48177755 [Truhan D.I., Filimonov S.N. Differencial'nyj diagnoz osnovnyh simptomov i sindromov pri zabolevaniyah zheludochno-kishechnogo trakta. Novokuzneck: Poligrafist, 2022. URL: https://elibrary.ru/ item.asp?id=48177755 (in Russian).]
62. Hirschfield GM, Chazouillères O, Cortez-Pinto H et al. A consensus integrated care pathway for patients with primary biliary cholangitis: a guideline-based approach to clinical care of patients. Expert Rev Gastroenterol Hepatol 2021; 15 (8): 929–39.
63. Lebrec D, Sicot C, Degott C, Benhamou JP. Portal hypertension and primary biliary cirrhosis. Digestion 1976; 14 (3): 220–6. DOI: 10.1159/000197934
64. Huet PM, Vincent C, Deslauriers J et al. Portal hypertension in primary biliary cirrhosis (PBC): A reversible condition? Yes, but not in all UDCA treated patients. Hepatol Res 2009; 39 (10): 1032–8. DOI: 10.1111/j.1872-034X.2009.00550.x
65. Warnes TW, Roberts SA, Smith A et al. Portal hypertension in primary biliary cholangitis: prevalence, natural history and histological correlates. Eur J Gastroenterol Hepatol 2021; 33 (12): 1595–602. DOI: 10.1097/MEG.0000000000002033
66. Lindor KD, Gershwin ME, Poupon R et al. Primary biliary cirrhosis. Hepatology 2009; 50 (1): 291–308. DOI: 10.1002/hep.22906
67. Assis DN. Chronic Complications of Cholestasis: Evaluation and Management. Clin Liver Dis 2018; 22 (3): 533–44. DOI: 10.1016/ j.cld.2018.03.014
68. Quarneti C, Muratori P, Lalanne C et al. Fatigue and pruritus at onset identify a more aggressive subset of primary biliary cirrhosis. Liver Int 2015; 35 (2): 636–41. DOI: 10.1111/liv.12560
69. Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med 2005; 353 (12): 1261–73. DOI: 10.1056/NEJMra043898
70. Yamagiwa S, Kamimura H, Takamura M, Aoyagi Y. Autoantibodies in primary biliary cirrhosis: recent progress in research on the pathogenetic and clinical significance. World J Gastroenterol 2014; 20 (10): 2606–12. DOI: 10.3748/wjg.v20.i10.2606
71. Murillo Perez CF, Goet JC, Lammers WJ et al. Milder disease stage in patients with primary biliary cholangitis over a 44-year period: A changing natural history. Hepatology 2018; 67 (5): 1920–30. DOI: 10.1002/hep.29717
72. Janmohamed A, Trivedi PJ. Patterns of disease progression and incidence of complications in primary biliary cholangitis (PBC). Best Pract Res Clin Gastroenterol 2018; 34–35: 71–83. DOI: 10.1016/ j.bpg.2018.06.002
73. Invernizzi P, Crosignani A, Battezzati PM et al. Comparison of the clinical features and clinical course of antimitochondrial antibodypositive and -negative primary biliary cirrhosis. Hepatology 1997; 25 (5): 1090–5. DOI: 10.1002/hep.510250507
74. Granito A, Muratori P, Muratori L et al. Antinuclear antibodies giving the ‘multiple nuclear dots’ or the ‘rim-like/membranous’ patterns: diagnostic accuracy for primary biliary cirrhosis. Aliment Pharmacol Ther 2006; 24 (11–2): 1575–83. DOI: 10.1111/j.1365- 2036.2006.03172.x
75. Granito A, Muratori P, Quarneti C et al. Antinuclear antibodies as ancillary markers in primary biliary cirrhosis. Expert Rev Mol Diagn 2012; 12 (1): 65–74. DOI: 10.1586/erm.11.82
76. Nakamura M, Kondo H, Mori T et al. Anti-gp210 and anti-centromere antibodies are different risk factors for the progression of primary biliary cirrhosis. Hepatology 2007; 45 (1): 118–27. DOI: 10.1002/hep.21472
77. Mahl TC, Shockcor W, Boyer JL. Primary biliary cirrhosis: survival of a large cohort of symptomatic and asymptomatic patients followed for 24 years. J Hepatol 1994; 20 (6): 707–13. DOI: 10.1016/s0168-8278(05)80139-4
78. Metcalf JV, Mitchison HC, Palmer JM et al. Natural history of early primary biliary cirrhosis. Lancet 1996; 348 (9039): 1399–402. DOI: 10.1016/S0140-6736(96)04410-8
79. Prince M, Chetwynd A, Newman W et al. Survival and symptom progression in a geographically based cohort of patients with primary biliary cirrhosis: follow-up for up to 28 years. Gastroenterology 2002; 123 (4): 1044–51. DOI: 10.1053/gast.2002.36027 58 | КЛИНИЧЕСКИЙ РАЗБОР В ОБщЕЙ МЕДИЦИНЕ | ТОМ 4 | №2 | 2023 | Clinical review for general practice | vol. 4 | no. 2 | 2023 | Обзор / Review
80. Locke GR, Therneau TM, Ludwig J et al. Time course of histological progression in primary biliary cirrhosis. Hepatology 1996; 23 (1): 52–6. DOI: 10.1002/hep.510230108
81. Boonstra K, Beuers U, Ponsioen CY. Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: A systematic review. J Hepatol 2012; 56 (5): 1181–8. DOI: 10.1016/j.jhep.2011.10.025
82. Bowlus CL, Gershwin ME. The Diagnosis of Primary Biliary Cirrhosis. Autoimmun Rev 2014; 13 (4–5): 441–4. DOI: 10.1016/j.autrev. 2014.01.041
83. Ruiz A, Lemoinne S, Carrat F et al. Radiologic course of primary sclerosing cholangitis: assessment by three-dimensional magnetic resonance cholangiography and predictive features of progression. Hepatology 2014; 59 (1): 242–50. DOI: 10.1002/hep.26620
84. Rawashdeh B, Couillard A, Rawshdeh A et al. Primary Biliary Cholangitis: A Mini-review. Gene Expression 2022; 21 (2): 34–40. DOI: 10.14218/GE.2022.00016
85. Haliloglu N, Erden A, Erden I. Primary biliary cirrhosis: evaluation with T2-weighted MR imaging and MR cholangiopancreatography. Eur J Radiol 2009; 69 (3): 523–7. DOI: 10.1016/j.ejrad.2007.11.003
86. Sy AM, Ferreira RD, John BV. Hepatocellular Carcinoma in Primary Biliary Cholangitis. Clin Liver Dis 2022; 26 (4): 691–704. DOI: 10.1016/j.cld.2022.06.011
87. McGee EE, Castro FA, Engels EA et al. Associations between autoimmune conditions and hepatobiliary cancer risk among elderly US adults. Int J Cancer 2019; 144 (4): 707–17. DOI: 10.1002/ijc.31835
88. Natarajan Y, Tansel A, Patel P et al. Incidence of Hepatocellular Carcinoma in Primary Biliary Cholangitis: A Systematic Review and Meta-Analysis. Dig Dis Sci 2021; 66 (7): 2439–51. DOI: 10.1007/s10620-020-06498-7
89. Rong G, Wang H, Bowlus CL et al. Incidence and risk factors for hepatocellular carcinoma in primary biliary cirrhosis. Clin Rev Allergy Immunol 2015; 48 (2–3): 132–41. DOI: 10.1007/s12016-015-8483-x
90. Trauner M, Graziadei IW. Review article: mechanisms of action and therapeutic applications of ursodeoxycholic acid in chronic liver diseases. Aliment Pharmacol Ther 1999; 13 (8): 979–96 DOI: 10.1046/j.1365-2036.1999.00596.x
91. Harms MH, van Buuren HR, Corpechot C et al. Ursodeoxycholic acid therapy and liver transplant-free survival in patients with primary biliary cholangitis. J Hepatol 2019; 71 (2): 357–65.
92. Achufusi TGO, Safadi AO, Mahabadi N. Ursodeoxycholic Acid. StatPearls Publishing, 2022. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan. 2022 Jul 25. URL: https: //pubmed.ncbi.nlm.nih.gov/31424887/
93. Patel A, Seetharam A. Primary Biliary Cholangitis: Disease Pathogenesis and Implications for Established and Novel Therapeutics. J Clin Exp Hepatol 2016; 6 (4): 311–8. DOI: 10.1016/j.jceh.2016.10.001
94. Bahar R, Wong KA, Liu CH, Bowlus CL. Update on New Drugs and Those in Development for the Treatment of Primary Biliary Cholangitis. Gastroenterol Hepatol (NY) 2018; 14 (3): 154–63. URL: https: //pubmed.ncbi.nlm.nih.gov/29928160/
95. Lammers WJ, Hirschfield GM, Corpechot C et al.; Global PBC Study Group. Development and Validation of a Scoring System to Predict Outcomes of Patients With Primary Biliary Cirrhosis Receiving Ursodeoxycholic Acid Therapy. Gastroenterology 2015; 149 (7): 1804– 1812.e4. DOI: 10.1053/j.gastro.2015.07.061
96. Boberg KM, Wisløff T, Kjøllesdal KS et al. Cost and health consequences of treatment of primary biliary cirrhosis with ursodeoxycholic acid. Aliment Pharmacol Ther 2013; 38 (7): 794–803. DOI: 10.1111/apt.12435
97. Cheung AC, Lammers WJ, Murillo Perez CF et al. Effects of Age and Sex of Response to Ursodeoxycholic Acid and Transplant-free Survival in Patients with Primary Biliary Cholangitis. Clin Gastroenterol Hepatol 2019; 17 (10): 2076–84.e2. DOI: 10.1016/j.cgh.2018.12.028
98. John BV, Aitcheson G, Schwartz KB et al. Male Sex Is Associated With Higher Rates of Liver-Related Mortality in Primary Biliary Cholangitis and Cirrhosis. Hepatology 2021; 74 (2): 879–91. DOI: 10.1002/hep.31776
99. Tang R, Wei Y, Li Y et al. Gut microbial profile is altered in primary biliary cholangitis and partially restored after UDCA therapy. Gut 2018; 67 (3): 534–41. DOI: 10.1136/gutjnl-2016-313332
100. John BV, Schwartz K, Levy C, Dahman B et al. Impact of Obeticholic acid Exposure on Decompensation and Mortality in Primary Biliary Cholangitis and Cirrhosis. Hepatol Commun 2021; 5 (8): 1426–36. DOI: 10.1002/hep4.1720
101. Kulkarni AV, Tevethia HV, Arab JP et al. Efficacy and safety of obeticholic acid in liver disease-A systematic review and meta-analysis. Clin Res Hepatol Gastroenterol 2021; 45 (3): 101675. DOI: 10.1016/j.clinre.2021.101675
102. Hirschfield GM, Mason A, Luketic V et al. Efficacy of obeticholic acid in patients with primary biliary cirrhosis and inadequate response to ursodeoxycholic acid. Gastroenterology 2015; 148 (4): 751–61.e8. DOI: 10.1053/j.gastro.2014.12.005
103. Hirschfield GM, Mason A, Luketic V et al. Efficacy of obeticholic acid in patients with primary biliary cirrhosis and inadequate response to ursodeoxycholic acid. Gastroenterology 2015; 148 (4): 751–61.e8. DOI: 10.1053/j.gastro.2014.12.005
104. Li Y, Lei R, Lei H et al. Side effect profile of pharmacologic therapies for liver fibrosis in nonalcoholic fatty liver disease: a systematic review and network meta-analysis. Eur J Gastroenterol Hepatol 2023; 35 (1): 1–14. DOI: 10.1097/MEG.0000000000002471
105. LiverTox: Clinical and Research Information on Drug-Induced Liver Injury [Internet]. Bethesda (MD): National Institute of Diabetes and Digestive and Kidney Diseases; 2012. URL: https://pubmed. ncbi.nlm.nih.gov/31643176/
106. Honda A, Ikegami T, Nakamuta M et al. Anticholestatic effects of bezafibrate in patients with primary biliary cirrhosis treated with ursodeoxycholic acid. Hepatology 2013; 57 (5): 1931–41. DOI: 10.1002/hep.26018
107. Vuppalanchi R, González-Huezo MS, Payan-Olivas R et al. A Multicenter, Open-Label, Single-Arm Study to Evaluate the Efficacy and Safety of Saroglitazar in Patients With Primary Biliary Cholangitis. Clin Transl Gastroenterol 2021; 12 (4): e00327. DOI: 10.14309/ ctg.0000000000000327
108. Ian Gan S, de Jongh M, Kaplan MM. Modafinil in the treatment of debilitating fatigue in primary biliary cirrhosis: a clinical experience. Dig Dis Sci 2009; 54 (10): 2242–6. DOI: 10.1007/s10620-008-0613-3
109. de Vries E, Bolier R, Goet J et al. Fibrates for Itch (FITCH) in Fibrosing Cholangiopathies: A Double-Blind, Randomized, Placebo-Controlled Trial. Gastroenterology 2021; 160 (3): 734–43. DOI: 10.1053/j.gastro.2020.10.001
110. Cançado GGL, Couto CA, Guedes LV et al. Fibrates for the Treatment of Primary Biliary Cholangitis Unresponsive to Ursodeoxycholic Acid: An Exploratory Study. Front Pharmacol 2022; 12: 818089. DOI: 10.3389/fphar.2021.818089
111. Ballantyne JC, Loach AB, Carr DB. Itching after epidural and spinal opiates. Pain 1988; 33 (2): 149–60. DOI: 10.1016/0304-3959 (88)90085-1
112. Liberal R, Zen Y, Mieli-Vergani G, Vergani D. Liver transplantation and autoimmune liver diseases. Liver Transpl 2013; 19 (10): 1065–77. DOI: 10.1002/lt.23704
113. Kwong AJ, Kim WR, Lake JR et al. OPTN/SRTR 2019 Annual Data Report: Liver. Am J Transplant 2021; 21 (Suppl. 2): 208–315. DOI: 10.1111/ajt.16494
114. Montano-Loza AJ, Hansen BE, Corpechot C et al. Factors Associated With Recurrence of Primary Biliary Cholangitis After Liver Transplantation and Effects on Graft and Patient Survival. Gastroenterology 2019; 156 (1): 96–107. DOI: 10.1053/j.gastro.2018.10.001
115. Bosch A, Dumortier J, Maucort-Boulch D et al. Preventive administration of UDCA after liver transplantation for primary biliary cirrhosis is associated with a lower risk of disease recurrence. J Hepatol 2015; 63 (6): 1449–58. DOI: 10.1016/j.jhep.2015.07.038
116. Kim WR, Lindor KD, Locke GR. Epidemiology and natural history of primary biliary cirrhosis in a US community. Gastroenterology 2000; 119 (6): 1631–6.
117. Parés A, Caballería L, Rodés J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic Acid. Gastroenterology 2006; 130 (3): 715–20. DOI: 10.1053/j.gastro.2005.12.029
118. 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 (6): 530–4. DOI: 10.1016/j.cgh.2010.03.004
119. Kimura N, Takamura M, Takeda N et al. Paris II and Rotterdam criteria are the best predictors of outcomes in patients with primary biliary cholangitis in Japan. Hepatol Int 2021; 15 (2): 437–43. DOI: 10.1007/s12072-021-10163-0
120. Kumagi T, Guindi M, Fischer SE et al. Baseline ductopenia and treatment response predict long-term histological progression in primary | КЛИНИЧЕСКИЙ РАЗБОР В ОБщЕЙ МЕДИЦИНЕ | ТОМ 4 | №2 | 2023 | Clinical review for general practice | vol. 4 | no. 2 | 2023 | 59 Обзор / Review biliary cirrhosis. Am J Gastroenterol 2010; 105 (10): 2186–94. DOI: 10.1038/ajg.2010.216
121. Murillo Perez CF, Harms MH, Lindor KD et al. Goals of Treatment for Improved Survival in Primary Biliary Cholangitis: Treatment Target Should Be Bilirubin Within the Normal Range and Normalization of Alkaline Phosphatase. Am J Gastroenterol 2020; 115 (7): 1066–74 DOI: 10.14309/ajg.0000000000000557
122. Goet JC, Murillo Perez CF, Harms MH et al.; GLOBAL PBC Study Group. A Comparison of Prognostic Scores (Mayo, UK-PBC, and GLOBE) in Primary Biliary Cholangitis. Am J Gastroenterol 2021; 116 (7): 1514–22. DOI: 10.14309/ajg.0000000000001285
123. Martin P, DiMartini A, Feng S et al. Evaluation for liver transplantation in adults: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation. Hepatology 2014; 59 (3): 1144–65. DOI: 10.1002/ hep.26972
124. Carbone M, Neuberger J. Liver transplantation in PBC and PSC: indications and disease recurrence. Clin Res Hepatol Gastroenterol 2011; 35 (6–7): 446–54. DOI: 10.1016/j.clinre.2011.02.007
125. Neuberger J, Portmann B, Macdougall BR et al. Recurrence of primary biliary cirrhosis after liver transplantation. N Engl J Med 1982; 306 (1): 1–4. DOI: 10.1056/NEJM198201073060101
126. ter Borg PC, Schalm SW, Hansen BE, van Buuren HR; Dutch PBC Study Group. Prognosis of ursodeoxycholic Acid-treated patients with primary biliary cirrhosis. Results of a 10-yr cohort study involving 297 patients. Am J Gastroenterol 2006; 101 (9): 2044–50. DOI: 10.1111/j.1572-0241.2006.00699.x
127. Mayo MJ. Natural history of primary biliary cirrhosis. Clin Liver Dis 2008; 12 (2): 277–88. DOI: 10.1016/j.cld.2008.02.012
Для цитирования:Трухан Д.И. Первичный билиарный холангит: новая дефиниция заболевания, актуальные аспекты диагностики и лечения. Обзор литературы. Клинический разбор в общей медицине. 2023; 4 (2): 48–59. DOI: 10.47407/kr2023.4.2.00204
Журнал предоставляет свободный доступ с возможностью использовать статьи в некоммерческих целях при условии указания авторства в рамках лицензии CC BY-NC-SA 4.0 (https://creativecommons.org/licenses/by-nc-sa/4.0/deed.ru)