Вариантная форма аутоиммунного поражения печени (первичный билиарный холангит/аутоиммунный гепатит-перекрест), ассоциированная с генерализованным саркоидозом (клинические наблюдения и обзор литературы)

Бурневич Э.З., Попова Е.Н., Пономарев А.Б. и др. Вариантная форма аутоиммунного поражения печени (первичный билиарный холангит/аутоиммунный гепатит-перекрест), ассоциированная с генерализованным саркоидозом (клинические наблюдения и обзор литературы). Терапевтический архив. 2019; 91 (1): 16–94.
DOI: 10.26442/00403660.2019.01.000036

________________________________________________

Burnevich E.S., Popova E.N., Ponomarev A.B. et al. Autoimmune liver disease (primary biliary cholangitis/autoimmune hepatitis-overlap) associated with sarcoidosis (clinical cases and literature review). Therapeutic Archive. 2019; 91 (1): 16–94.
DOI: 10.26442/00403660.2019.01.000036

Читать PDF

Аннотация

Приводятся клинические наблюдения сочетания генерализованного саркоидоза с поражением легких, внутригрудных лимфоузлов и кожи у больных с вариантной формой аутоиммунного поражения печени с подробным клинико-инструментально-морфологическим обследованием. На примере клинических случаев обсуждаются современные аспекты патогенеза гранулематозных заболеваний с аутоиммунными нарушениями.

Ключевые слова: саркоидоз, аутоиммунный гепатит, первичный билиарный холангит, первичный билиарный аутоиммунный холангит гепатит-перекрест, внепеченочные проявления.

________________________________________________

Clinical features of overlap autoimmune hepatitis/primary biliary cholangitis and morphological-proved sarcoid lesions (lungs, lymph nodes, skin) were performed. Data of long-term clinical observation presented in comparison with the results of laboratory datas, instrumental and morphological studies of liver tissue, lungs, skin. The modern aspects of pathogenesis of association autoimmune and granulomatous diseases arediscussed on the example of clinical cases of combination of cholestatic variants of autoimmune hepatitis and generalized sarcoidosis.

Keywords: sarcoidosis, autoimmune hepatitis, primary biliary cholangitis, primary biliary cholangitis-autoimmune hepatitis-overlap, extrahepatic manifestations.

Список литературы

1. Waldenstrom J. Blutproteine und Nahrungseiweiss. Dtsch Ges Verdau Stoffwechselkr. 1950;15:113-9. https://doi.org/10.1016/s0168-8278(02)00408-7
2. Mackay I, Taft L, Cowling D. Lupoid hepatitis. Lancet. 1956;271:1323-6. https://doi.org/10.1016/s0140-6736(56)91483-0
3. Mieli-Vergani G, Vergani D, Czaja A, Manns M, Krawitt E, Vierling J, Lohse A, Montano-Loza A. Autoimmune hepatitis. Nat Rev Dis Primers. 2018;4:18017. https://doi.org/10.1038/nrdp.2018.17
4. Ozaslan E, Efe C, Gokbulut Ozaslan N. The diagnosis of antimitochondrial antibody-negative primary biliary cholangitis. Clin Res Hepatol Gastroenterol. 2016;40(5):553-61. https://doi.org/10.1016/j.clinre.2016.06.001
5. Александрова Е.А., Авдеев В.Г., Бурневич Э.З., Арион Е.А. Первичный склерозирующий холангит – современные представления о патогенезе, диагностике, лечении. Терапевтический архив. 2012;8:86-90 [Aleksandrova E, Avdeev V, Burnevich E, Arion E. Primary sclerosing cholangitis – modern view on pathogenesis, diagnosys, treatment. Therapeutic Archive. 2012;8:86-90 (In Russ.)].
6. Dyson J, Beuers U, Jones D, Lohse A, Hudson M. Primary sclerosing cholangitis. Lancet. 2018;391(10139):2547-59. https://doi.org/10.1016/s0140-67 36(18)30300-3
7. Czaja A, Carpenter H. Autoimmune Hepatitis Overlap Syndromes and Liver Pathology. Gastroenterol Clin North Am. 2017;46(2):345-64. https://doi.org/10.1016/j.gtc.2017.01.008
8. Guo L, Zhou L, Zhang N, Deng B, Wang B. Extrahepatic Autoimmune Diseases in Patients with Autoimmune Liver Diseases: A Phenomenon Neglected by Gastroenterologists. Gastroenterol Res Pract. 2017;2017: 2376231. Published online 2017 Jan 16. doi: 10.1155/2017/2376231
9. Surani S, Mendez Y, Anjum H, Varon J. Pulmonary complications of hepatic diseases. World J Gastroenterol. 2016;22(26):6008-15. https://doi.org/10.3748/wjg.v22.i26.6008
10. Shoenfeld Y, Blank M, Abu-Shakra M, Amital H, Barzilai O, Berkun Y, Bizzaro N, Gilburd B, Zandman-Goddard G, Katz U, Krause I, Langevitz P, Mackay I, Orbach H, Ram M, Sherer Y, Toubi E, Gershwin M. The mosaic of autoimmunity: prediction, autoantibodies, and therapy in autoimmune diseases – 2008. Isr Med Assoc J. 2008;10(1):13-9.
11. Pamfil C, Candrea E, Berki E, Popov H, Radu P, Rednic S. Primary biliary cirrhosis--autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome. J Gastrointestin Liver Dis. 2015;24(1):101-4. https://doi.org/10.15403/jgld.201 4.1121.cpa
12. Aksoy E, Yilmaz B., Köklü S. Autoimmune hepatitis/primary biliary cirrhosis overlap syndrome developed in a patient with vitiligo and Hashimoto thyroiditis. Eur J Gastroenterol Hepatol. 2013;25(1):121-2. https://doi. org/10.10 97/meg.0b013e328359493e
13. Hutchinson J. Anomalous diseases of skin ad fingers; case of livid papillary psoriasis? In: Churchill J, Churchill A, eds. Illustrations of Clinical Surgery. London, 1877:42-3.
14. Bargagli E, Prasse A. Sarcoidosis: a review for the internist. Intern Emerg Med. 2018;13(3):325-31. https://doi.org/10.1007/s11739-017-1778-6
15. Корнев Б.М. Внелегочные проявления саркоидоза. Клиническая медицина. 1996;74(8):62-3 [Kornev B. Extrapulmonary manifestations of sarcoidosis. Klinicheskaya medicina. 1996;74(8):62-3 (In Russ.)].
16. Попова Е.Н., Стрижаков Л.А., Шоломова В.И., Пономарев А.Б., Моисеев С.В., Бровко М.Ю., Бондаренко И.Б., Пономарева Л.А., Фомин В.В. Клинические особенности поражения сердца при саркоидозе. Терапевтический архив. 2018;1:54-9 [Popova E, Strizhakov L, Sholomova V, Ponomarev A, Moiseev S, Brovko M, Bondarenko I, Ponomareva L, Fomin V. Clinical features of cardiac lesion in patients with generalized sarcoidosis. Therapeutic Archive. 2018;1:54-9 (In Russ.)]. https://doi.org/10.17116/ter arkh201890154-59
17. Darlington P, Gabrielsen A, Sörensson P, Tallstedt L, Padyukov L, Eklund A, Grunewald J. HLA-alleles associated with increased risk for extra-pulmonary involvement in sarcoidosis. Tissue Antigens. 2014;83(4):267-72. https://doi.org/ 10.1111/tan.12326
18. Medica I, Kastrin A, Maver A, Peterlin B. Role of genetic polymorphisms in ACE and TNF-alpha gene in sarcoidosis: a meta-analysis. J Hum Genet. 2007;52(10):836-47. https://doi.org/10.1007/s10038-007-0185-7
19. Oswald-Richter K, Sato H, Hajizadeh R, Shepherd B, Sidney J, Sette A, Newman L, Drake W. Mycobacterial ESAT-6 and katG are recognized by sarcoidosis CD4+ T cells when presented by the American sarcoidosis susceptibility allele, DRB1*1101. J Clin Immunol. 2010;30(1):157-66. https://doi.org/10.1007/s10875-009-9311-y
20. Darlington P, Tallstedt L, Padyukov L, Kockum I, Cederlund K, Eklund A, Grunewald J. HLA-DRB1* alleles and symptoms associated with Heerfordt's syndrome in sarcoidosis. Eur Respir J. 2011;38(5):1151-7. https://doi: 10.1183/ 09031936.00025011
21. Faurie P, Broussolle C, Zoulim F, Trepo C, Sève P. Sarcoidosis and hepatitis C: clinical description of 11 cases. Eur J Gastroenterol Hepatol. 2010;22(8):967-72. https://doi.org/10.1097/meg.0b013e32833834bc
22. Gayet A, Plaisance P, Bergmann J, Mouly S. Development of sarcoidosis following completion of treatment for hepatitis C with pegylated interferon-{alpha}2a and ribavirin: a case report and literature review. Clin Med Res. 2010;8(3-4):163-7. https://doi.org/10.3121/cmr.2010.886
23. Brjalin V, Salupere R, Tefanova V, Prikk K, Lapidus N, Jõeste E. Sarcoidosis and chronic hepatitis C: a case report. World J Gastroenterol. 2012;18(40):5816-20. https://doi.org/10.3748/wjg.v18.i40.5816
24. Willoughby J. Crohn's disease and sarcoidosis in siblings: follow-up of a published report with a new case and brief review of the literature. Frontline Gastroenterol. 2017;8(1):74-7. https://doi.org/10.1136/flgastro-2015-100666
25. Wagner J, Skinner N, Catto-Smith A, Cameron D, Michalski W, Visvanathan K, Kirkwood C. TLR4, IL10RA, and NOD2 mutation in paediatric Crohn's disease patients: an association with Mycobacterium avium subspecies paratuberculosis and TLR4 and IL10RA expression. Med Microbiol Immunol. 2013;202(4):267-76. https://doi.org/10.1007/s00430-013-0290-5
26. Strober W, Asano N, Fuss I, Kitani A, Watanabe T. Cellular and molecular mechanisms underlying NOD2 risk-associated polymorphisms in Crohn's disease. Immunol Rev. 2014;260(1):249-60. https://doi.org/10.1111/imr.12193
27. Kishor S, Turner M, Borg B, Kleiner D, Cowen E. Cutaneous sarcoidosis and primary biliary cirrhosis: A chance association or related diseases? J Am Acad Dermatol. 2008;58(2):326-35. https://doi.org/10.1016/j.jaad.2007.07.031
28. Cerri S, du Bois R, Spagnolo P. Genetic commonality between inflammatory bowel disease and sarcoidosis: the beginning of the end or the end of the beginning? Eur Respir J. 2011;37(3):489-91.

________________________________________________

1. Waldenstrom J. Blutproteine und Nahrungseiweiss. Dtsch Ges Verdau Stoffwechselkr. 1950;15:113-9. https://doi.org/10.1016/s0168-8278(02)00408-7
2. Mackay I, Taft L, Cowling D. Lupoid hepatitis. Lancet. 1956;271:1323-6. https://doi.org/10.1016/s0140-6736(56)91483-0
3. Mieli-Vergani G, Vergani D, Czaja A, Manns M, Krawitt E, Vierling J, Lohse A, Montano-Loza A. Autoimmune hepatitis. Nat Rev Dis Primers. 2018;4:18017. https://doi.org/10.1038/nrdp.2018.17
4. Ozaslan E, Efe C, Gokbulut Ozaslan N. The diagnosis of antimitochondrial antibody-negative primary biliary cholangitis. Clin Res Hepatol Gastroenterol. 2016;40(5):553-61. https://doi.org/10.1016/j.clinre.2016.06.001
5. Aleksandrova E, Avdeev V, Burnevich E, Arion E. Primary sclerosing cholangitis – modern view on pathogenesis, diagnosys, treatment. Therapeutic Archive. 2012;8:86-90 (In Russ.)
6. Dyson J, Beuers U, Jones D, Lohse A, Hudson M. Primary sclerosing cholangitis. Lancet. 2018;391(10139):2547-59. https://doi.org/10.1016/s0140-67 36(18)30300-3
7. Czaja A, Carpenter H. Autoimmune Hepatitis Overlap Syndromes and Liver Pathology. Gastroenterol Clin North Am. 2017;46(2):345-64. https://doi.org/10.1016/j.gtc.2017.01.008
8. Guo L, Zhou L, Zhang N, Deng B, Wang B. Extrahepatic Autoimmune Diseases in Patients with Autoimmune Liver Diseases: A Phenomenon Neglected by Gastroenterologists. Gastroenterol Res Pract. 2017;2017: 2376231. Published online 2017 Jan 16. doi: 10.1155/2017/2376231
9. Surani S, Mendez Y, Anjum H, Varon J. Pulmonary complications of hepatic diseases. World J Gastroenterol. 2016;22(26):6008-15. https://doi.org/10.3748/wjg.v22.i26.6008
10. Shoenfeld Y, Blank M, Abu-Shakra M, Amital H, Barzilai O, Berkun Y, Bizzaro N, Gilburd B, Zandman-Goddard G, Katz U, Krause I, Langevitz P, Mackay I, Orbach H, Ram M, Sherer Y, Toubi E, Gershwin M. The mosaic of autoimmunity: prediction, autoantibodies, and therapy in autoimmune diseases – 2008. Isr Med Assoc J. 2008;10(1):13-9.
11. Pamfil C, Candrea E, Berki E, Popov H, Radu P, Rednic S. Primary biliary cirrhosis--autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome. J Gastrointestin Liver Dis. 2015;24(1):101-4. https://doi.org/10.15403/jgld.201 4.1121.cpa
12. Aksoy E, Yilmaz B., Köklü S. Autoimmune hepatitis/primary biliary cirrhosis overlap syndrome developed in a patient with vitiligo and Hashimoto thyroiditis. Eur J Gastroenterol Hepatol. 2013;25(1):121-2. https://doi. org/10.10 97/meg.0b013e328359493e
13. Hutchinson J. Anomalous diseases of skin ad fingers; case of livid papillary psoriasis? In: Churchill J, Churchill A, eds. Illustrations of Clinical Surgery. London, 1877:42-3.
14. Bargagli E, Prasse A. Sarcoidosis: a review for the internist. Intern Emerg Med. 2018;13(3):325-31. https://doi.org/10.1007/s11739-017-1778-6
15. Kornev B. Extrapulmonary manifestations of sarcoidosis. Klinicheskaya medicina. 1996;74(8):62-3 (In Russ.)
16. Popova E, Strizhakov L, Sholomova V, Ponomarev A, Moiseev S, Brovko M, Bondarenko I, Ponomareva L, Fomin V. Clinical features of cardiac lesion in patients with generalized sarcoidosis. Therapeutic Archive. 2018;1:54-9 (In Russ.)
https://doi.org/10.17116/ter arkh201890154-59
17. Darlington P, Gabrielsen A, Sörensson P, Tallstedt L, Padyukov L, Eklund A, Grunewald J. HLA-alleles associated with increased risk for extra-pulmonary involvement in sarcoidosis. Tissue Antigens. 2014;83(4):267-72. https://doi.org/ 10.1111/tan.12326
18. Medica I, Kastrin A, Maver A, Peterlin B. Role of genetic polymorphisms in ACE and TNF-alpha gene in sarcoidosis: a meta-analysis. J Hum Genet. 2007;52(10):836-47. https://doi.org/10.1007/s10038-007-0185-7
19. Oswald-Richter K, Sato H, Hajizadeh R, Shepherd B, Sidney J, Sette A, Newman L, Drake W. Mycobacterial ESAT-6 and katG are recognized by sarcoidosis CD4+ T cells when presented by the American sarcoidosis susceptibility allele, DRB1*1101. J Clin Immunol. 2010;30(1):157-66. https://doi.org/10.1007/s10875-009-9311-y
20. Darlington P, Tallstedt L, Padyukov L, Kockum I, Cederlund K, Eklund A, Grunewald J. HLA-DRB1* alleles and symptoms associated with Heerfordt's syndrome in sarcoidosis. Eur Respir J. 2011;38(5):1151-7. https://doi: 10.1183/ 09031936.00025011
21. Faurie P, Broussolle C, Zoulim F, Trepo C, Sève P. Sarcoidosis and hepatitis C: clinical description of 11 cases. Eur J Gastroenterol Hepatol. 2010;22(8):967-72. https://doi.org/10.1097/meg.0b013e32833834bc
22. Gayet A, Plaisance P, Bergmann J, Mouly S. Development of sarcoidosis following completion of treatment for hepatitis C with pegylated interferon-{alpha}2a and ribavirin: a case report and literature review. Clin Med Res. 2010;8(3-4):163-7. https://doi.org/10.3121/cmr.2010.886
23. Brjalin V, Salupere R, Tefanova V, Prikk K, Lapidus N, Jõeste E. Sarcoidosis and chronic hepatitis C: a case report. World J Gastroenterol. 2012;18(40):5816-20. https://doi.org/10.3748/wjg.v18.i40.5816
24. Willoughby J. Crohn's disease and sarcoidosis in siblings: follow-up of a published report with a new case and brief review of the literature. Frontline Gastroenterol. 2017;8(1):74-7. https://doi.org/10.1136/flgastro-2015-100666
25. Wagner J, Skinner N, Catto-Smith A, Cameron D, Michalski W, Visvanathan K, Kirkwood C. TLR4, IL10RA, and NOD2 mutation in paediatric Crohn's disease patients: an association with Mycobacterium avium subspecies paratuberculosis and TLR4 and IL10RA expression. Med Microbiol Immunol. 2013;202(4):267-76. https://doi.org/10.1007/s00430-013-0290-5
26. Strober W, Asano N, Fuss I, Kitani A, Watanabe T. Cellular and molecular mechanisms underlying NOD2 risk-associated polymorphisms in Crohn's disease. Immunol Rev. 2014;260(1):249-60. https://doi.org/10.1111/imr.12193
27. Kishor S, Turner M, Borg B, Kleiner D, Cowen E. Cutaneous sarcoidosis and primary biliary cirrhosis: A chance association or related diseases? J Am Acad Dermatol. 2008;58(2):326-35. https://doi.org/10.1016/j.jaad.2007.07.031
28. Cerri S, du Bois R, Spagnolo P. Genetic commonality between inflammatory bowel disease and sarcoidosis: the beginning of the end or the end of the beginning? Eur Respir J. 2011;37(3):489-91.

Авторы

Э.З. Бурневич1,3, Е.Н. Попова1, А.Б. Пономарев2, Т.П. Некрасова2, М.В. Лебедева1, А.Л. Филатова1, С.Е. Щаницына1, Л.А. Пономарева1, В.Д. Бекетов1, И.Б. Бондаренко1, Е.Л. Танащук1, Е.Н. Никулкина1, С.В. Моисеев1

1 Кафедра внутренних, профессиональных болезней и ревматологии медико-профилактического факультета ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Москва, Россия;
2 Кафедра патологической анатомии им. акад. А.И. Струкова ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Москва, Россия;
3 ГБУЗ «Городская клиническая больница №24» Департамента здравоохранения г. Москвы, Москва, Россия

________________________________________________

E.S. Burnevich1,3, E.N. Popova1, A.B. Ponomarev2, T.P. Nekrasova2, M.V. Lebedeva1, A.L. Filatova1, E.M. Shchanitcyna1, L.A. Ponomareva1, V.D. Beketov1, I.B. Bondarenko1, E.L. Tanashchuk1, E.N. Nikulkina1, S.V. Moiseev1

1 Department of internal, occupational diseases and rheumatology, I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Moscow, Russia;
2 Department of pathological anatomy, I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Moscow, Russia;
3 City Clinical Hospital №24 of the Department of Health of Moscow, Moscow, Russia

Назад к списку

Цель портала OmniDoctor – предоставление профессиональной информации врачам, провизорам и фармацевтам.