Сочетание саркоидоза и первичного билиарного холангита у пациентки с синдромом холестаза

Бровко М.Ю., Акулкина Л.А., Калашников М.В. и др. Сочетание саркоидоза и первичного билиарного холангита у пациентки с синдромом холестаза. Терапевтический архив. 2021; 93 (2): 193–198. DOI: 10.26442/00403660.2021.02.200620

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Brovko M.Yu., Akulkina L.A., Kalashnikov M.V., et al Sarcoidosis and primary biliary cholangitis in a patient with cholestasis. Terapevticheskii Arkhiv (Ter. Arkh.). 2021; 93 (2): 193–198. DOI: 10.26442/00403660.2021.02.200620

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Аннотация

Первичный билиарный холангит и саркоидоз относятся к группе заболеваний печени, протекающих с преобладанием синдрома холестаза. Дифференциальная диагностика этих двух патологий основывается на совокупности данных анамнеза, а также объективного, лабораторного и инструментального обследования. Представленное наблюдение демонстрирует редкое сочетание у пациентки морфологически верифицированного поражения печени в рамках первичного билиарного холангита и саркоидоза. Несмотря на схожесть клинических проявлений, подходы к терапии данных заболеваний кардинально отличаются друг от друга, что указывает на необходимость исключения сочетанного поражения печени в сложных диагностических случаях.

Ключевые слова: первичный билиарный холангит, саркоидоз, холестаз

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Primary biliary cholangitis and sarcoidosis are both cholestatic liver diseases. Currently, there are no established specific criteria for distinguishing the diseases from each other; diagnosis is based on the anamnesis, as well as the results of physical, laboratory and instrumental examination. The case report presents a female patient with a rare combination of histologically verified liver sarcoidosis and primary biliary cholangitis. Despite the similar clinical manifestations, the approaches to the treatment of these diseases are completely different, that underlines the importance of the differential diagnosis to exclude combined liver damage.

Keywords: primary biliary cholangitis, sarcoidosis, cholestasis

Список литературы

1. Hirschfield GM, et al. 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
2. Valeyre D, Prasse A, NunesHet al. Sarcoidosis. Lancet. 2014;383(9923):1155-67. doi: 10.1016/S0140-6736(13)60680-7
3. Саркоидоз. Клинические рекомендации. Под ред. Н.А. Мухина. М.: ИМА-пресс, 2009; с. 54 [Sarkoidoz. Klinicheskie rekomendatsii. Pod red. N.A. Mukhina. Moscow: IMA-PRESS, 2009; p. 54 (In Russ.)].
4. Rajoriya N, Wotton CJ, Yeates DG, et al. Immune-mediated and chronic inflammatory disease in people with sarcoidosis: disease associations in a large UK database. Postgrad Med J. 2009;85(2003):233-7. doi: 10.1136/pgmj.2008.067769
5. Kishor S, Turner ML, Borg BB, et al. Cutaneous sarcoidosis and primary biliary cirrhosis: A chance association or related diseases? J Am Acad Dermatol. 2008;58(2):326-35. doi: 10.1016/j.jaad.2007.07.031
6. Hughes P, McGavin CR. Sarcoidosis and primary biliary cirrhosis with co-existing myositis. Thorax. 1997;52(2):201-2. doi: 10.1136/thx.52.2.201
7. Stanca CM, Fiel MI, Allina J, et al. Liver failure in an antimitochondrial antibody-positive patient with sarcoidosis: primary biliary cirrhosis or hepatic sarcoidosis? Semin Liver Dis. 2005;25(3):364-70. doi: 10.1055/s-2005-916327
8. Karlish AJ, Thompson RP, Williams R. A case of sarcoidosis and primary biliary cirrhosis. Lancet. 1969;2(7620):599. doi: 10.1016/s0140-6736(69)90306-7
9. Rudzki C, Ishak KG, Zimmerman HJ. Chronic intrahepatic cholestasis of sarcoidosis. Am J Med. 1975;59(3):373-87. doi: 10.1016/0002-9343(75)90396-4
10. Fagan EA, Moore-Gillon JC, Turner-Warwick M. Multiorgan granulomas and mitochondrial antibodies. N EngI J Med. 1983;308(10):572-5. doi: 10.1056/NEJM198303103081006
11. Zissel G, Prasse A., Müller-Quernheim J. Immunologic response of sarcoidosis. Semin Respir Crit Care Med. 2010;31(4):390-403. doi: 10.1055/s-0030-1262208
12. Holmes J, Lazarus A. Sarcoidosis: extrathoracic manifestations. Dis Mon. 2009;55:675-92. doi: 10.1016/j.disamonth.2009.05.002
13. Alempijević T, Sokić-Milutinović A, Toncev L, et al. Primary biliary cirrhosis and hepatic sarcoidosis – a case report. Vojnosanit Pregl. 2014;71(1):83-6. doi: 10.2298/vsp1401083a
14. Karagiannidis A, Karavalaki M, Koulaouzidis A. Hepaticsarcoidosis. Ann Hepatol. 2006;5(4):251-6. doi: 10.1016/S1665-2681(19)31983-0
15. Prince MI, 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;123:1044-51. doi: 10.1053/gast.2002.36027
16. Syed U, Alkhawam H, Bakhit M, et al. Hepatic sarcoidosis: pathogenesis, clinical context, and treatment options. Scand J Gastroenterol. 2016;51(9):1025-30. doi: 10.1080/00365521.2016.1177856
17. Farouj NE, Cadranel JF D, Mofredj A, et al. Ductopenia related liver sarcoidosis. World J Hepatol. 2011;3(6):170-4. doi: 10.4254/wjh.v3.i6.170
18. 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
19. Ayyala U, Padilla M. Diagnosis and treatment of hepatic sarcoidosis. Curr Treat Options Gastroenterol. 2006;9(6):475-83. doi: 10.1007/s11938-006-0004-9
20. Martusewicz-Boros MM, Boros PW, Wiatr E. Respiratory system involvement in chronic liver diseases. Pol Arch Med Wewn. 2013;123:635-42. doi: 10.20452/pamw.1980
21. Koksal D, Koksal AS, Gurakar A. Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis. J Clin Transl Hepatol. 2016;4:258-62. doi: 10.14218/JCTH.2016.00024

________________________________________________

1. Hirschfield GM, et al. 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
2. Valeyre D, Prasse A, NunesHet al. Sarcoidosis. Lancet. 2014;383(9923):1155-67. doi: 10.1016/S0140-6736(13)60680-7
3. Sarkoidoz. Klinicheskie rekomendatsii. Pod red. N.A. Mukhina. Moscow: IMA-PRESS, 2009; p. 54 (In Russ.)
4. Rajoriya N, Wotton CJ, Yeates DG, et al. Immune-mediated and chronic inflammatory disease in people with sarcoidosis: disease associations in a large UK database. Postgrad Med J. 2009;85(2003):233-7. doi: 10.1136/pgmj.2008.067769
5. Kishor S, Turner ML, Borg BB, et al. Cutaneous sarcoidosis and primary biliary cirrhosis: A chance association or related diseases? J Am Acad Dermatol. 2008;58(2):326-35. doi: 10.1016/j.jaad.2007.07.031
6. Hughes P, McGavin CR. Sarcoidosis and primary biliary cirrhosis with co-existing myositis. Thorax. 1997;52(2):201-2. doi: 10.1136/thx.52.2.201
7. Stanca CM, Fiel MI, Allina J, et al. Liver failure in an antimitochondrial antibody-positive patient with sarcoidosis: primary biliary cirrhosis or hepatic sarcoidosis? Semin Liver Dis. 2005;25(3):364-70. doi: 10.1055/s-2005-916327
8. Karlish AJ, Thompson RP, Williams R. A case of sarcoidosis and primary biliary cirrhosis. Lancet. 1969;2(7620):599. doi: 10.1016/s0140-6736(69)90306-7
9. Rudzki C, Ishak KG, Zimmerman HJ. Chronic intrahepatic cholestasis of sarcoidosis. Am J Med. 1975;59(3):373-87. doi: 10.1016/0002-9343(75)90396-4
10. Fagan EA, Moore-Gillon JC, Turner-Warwick M. Multiorgan granulomas and mitochondrial antibodies. N EngI J Med. 1983;308(10):572-5. doi: 10.1056/NEJM198303103081006
11. Zissel G, Prasse A., Müller-Quernheim J. Immunologic response of sarcoidosis. Semin Respir Crit Care Med. 2010;31(4):390-403. doi: 10.1055/s-0030-1262208
12. Holmes J, Lazarus A. Sarcoidosis: extrathoracic manifestations. Dis Mon. 2009;55:675-92. doi: 10.1016/j.disamonth.2009.05.002
13. Alempijević T, Sokić-Milutinović A, Toncev L, et al. Primary biliary cirrhosis and hepatic sarcoidosis – a case report. Vojnosanit Pregl. 2014;71(1):83-6. doi: 10.2298/vsp1401083a
14. Karagiannidis A, Karavalaki M, Koulaouzidis A. Hepaticsarcoidosis. Ann Hepatol. 2006;5(4):251-6. doi: 10.1016/S1665-2681(19)31983-0
15. Prince MI, 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;123:1044-51. doi: 10.1053/gast.2002.36027
16. Syed U, Alkhawam H, Bakhit M, et al. Hepatic sarcoidosis: pathogenesis, clinical context, and treatment options. Scand J Gastroenterol. 2016;51(9):1025-30. doi: 10.1080/00365521.2016.1177856
17. Farouj NE, Cadranel JF D, Mofredj A, et al. Ductopenia related liver sarcoidosis. World J Hepatol. 2011;3(6):170-4. doi: 10.4254/wjh.v3.i6.170
18. 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
19. Ayyala U, Padilla M. Diagnosis and treatment of hepatic sarcoidosis. Curr Treat Options Gastroenterol. 2006;9(6):475-83. doi: 10.1007/s11938-006-0004-9
20. Martusewicz-Boros MM, Boros PW, Wiatr E. Respiratory system involvement in chronic liver diseases. Pol Arch Med Wewn. 2013;123:635-42. doi: 10.20452/pamw.1980
21. Koksal D, Koksal AS, Gurakar A. Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis. J Clin Transl Hepatol. 2016;4:258-62. doi: 10.14218/JCTH.2016.00024

Авторы

М.Ю. Бровко1, Л.А. Акулкина1, М.В. Калашников1, Т.П. Розина1,2, Т.П. Некрасова1, В.И. Шоломова1,2, Д.В. Коновалов1, А.Ш. Янакаева1, С.В. Моисеев1,2

1 ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Москва, Россия;
2 ФГБОУ ВО «Московский государственный университет им. М.В. Ломоносова», Москва, Россия

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M.Yu. Brovko1, L.A. Akulkina1, M.V. Kalashnikov1, T.P. Rozina1,2, T.P. Nekrasova1, V.I. Sholomova1,2, D.V. Konovalov1, A.Sh. Yanakayeva1, S.V. Moiseev1,2

1 Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia;
2 Lomonosov Moscow State University, Moscow, Russia

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