Варианты поражения легких при аутоиммунных заболеваниях печени

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Аннотация

К первичным аутоиммунным заболеваниям печени традиционно относят первичный билиарный холангит, первичный склерозирующий холангит и аутоиммунный гепатит. Несмотря на преимущественное поражение органов гепатобилиарной системы, в течение последних десятилетий получено множество данных, свидетельствующих в пользу наличия внепеченочных проявлений данных заболеваний, в частности, поражения легких в виде очаговых и интерстициальных изменений с возможным прогрессированием, развитием фиброза и дыхательной недостаточности. При поражении легких в патологический процесс могут вовлекаться как паренхима органа, так и сосуды, плевра, внутригрудные лимфатические узлы, при этом наиболее достоверным методом, позволяющим оценить объем поражения в дебюте заболевания и его эволюцию в динамике, является компьютерная томография высокого разрешения. В связи с возможностью длительного бессимптомного течения легочного процесса с развитием необратимых изменений у пациентов с аутоиммунными заболеваниями печени представляется обоснованным проведение скрининговых исследований для своевременного выявления и лечения поражений легких в рамках данной патологии.

Ключевые слова: интерстициальные заболевания легких, первичный билиарный холангит, первичный склерозирующий холангит, аутоиммунный гепатит.

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The primary autoimmune liver diseases conventionally include primary biliary cholangitis, primary sclerosing cholangitis and autoimmune hepatitis. Despite of primary autoimmune affection of different parts of the hepatobiliary system, in the recent decades, a lot of data has emerged indicating the presence of extrahepatic manifestations of these diseases, in particular, lung lesions, such as nodular and interstitial changes with possible progression and development of fibrosis and respiratory failure. In case of lungs disease, both pulmonary parenchyma and lung vessels, pleura, and intrathoracic lymph nodes can be involved. The most sensitive and specific procedure to assess the extent of the lung lesions and their evolution is high-resolution computed tomography. Due to the possibility of long-term asymptomatic course of the pulmonary disease with development of irreversible changes in patients with autoimmune liver diseases, it seems reasonable to conduct screening studies aimed at early detection and treatment of lung lesions in this population.

Keywords: interstitial lung disease, primary biliary cholangitis, primary sclerosing cholangitis, autoimmune hepatitis.

Список литературы

1. Martusewicz-Boros MM, Boros PW, Wiatr E. Respiratory system involvement in chronic liver diseases. Pol Arch Med Wewn. 2013; 123:635-642. doi: 10.20452/pamw.1980
2. Lv X, Gao F, Liu Q, Zhang S, Huang Z, Zhu Y, Zong H, Li Q, Li S. Clinical and pathological characteristics of IgG4-related interstitial lung disease. Exp Ther Med. 2018;15:1465-1473. doi: 10.3892/etm. 2017.5554
3. Al-Mujaini A, Al-Khabori M, Shenoy K, Wali U. Immunoglobulin
G4-related disease: An update. Oman Med J. 2018;33:97-103.
doi: 10.5001/omj.2018.20
4. Ramachandraiah V, Aronow W, Chandy D. Pulmonary sarcoidosis:
an update. Postgrad Med Taylor & Francis. 2017;129:149-158.
doi: 10.1080/00325481.2017.1251818
5. Mells GF. Primary biliary cirrhosis: Family, genes, and bugs. Clin Liver Dis. 2014;3:69-73. doi: 10.1002/cld.308
6. Shen M, Zhang F, Zhang X. Primary Biliary Cirrhosis Complicated With Interstitial Lung Disease: A Prospective Study in 178 Patients.
J Clin Gastroenterol. 2009;43:676-679. doi: 10.1093/innovait/inq138
7. Koksal D, Koksal AS, Gurakar A. Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis. J Clin Transl Hepatol. 2016;4:258-262. doi: 10.14218/JCTH.2016.00024
8. Rodriguez-Roisin R, Pares A, Bruguera M, Coll J, Picado C. Pulmonary involvement in primary biliary cirrhosis. Thorax. 1981;36: 208-212.
9. Uddenfeldt P, Bjerle P, Danielsson A, Nyström L. Lung Function Abnormalities in Patients with Primary Biliary Cirrhosis. Acta Med Scand. 1988;223:549-555.
10. Krowka MJ, Grambsch PM, Edell ES, Cortese DA, Dickson ER. Primary biliary cirrhosis: relation between hepatic function and pulmonary function in patients who never smoked. Hepatology. 1991;13:1095-1100.
11. Costa C, Sambataro A, Baldi S, Modena V, Todros L, Libertucci D, Coni F, Fusaro E, Revello F, Murgia A. Primary biliary cirrhosis: lung involvement. Liver. 1995;15:196-201.
12. Wallaert B, Bonniere P, Prin L, Cortot A, Tonnel AB, Voisin C. Primary biliary cirrhosis. Subclinical inflammatory alveolitis in patients with normal chest roentgenograms. Chest. 1986;90:842-848.
13. Spiteri MA, Johnson M, Epstein O, Sherlock S, Clarke SW, Poulter LW. Immunological features of lung lavage cells from patients with primary biliary cirrhosis may reflect those seen in pulmonary sarcoidosis. Gut. 1990;31:208-212.
14. Kuś J, Bartosiewicz M, Siemion-Szcześniak I, Jędrych M, Radwan-Röhrenschef P, Lewandowska K, Langfort R, Oniszh K, Franczuk M, Kuś J, Wesołowski S. Interstitial lung disease in patients with primary biliary cirrhosis. Pneumonol Alergol Pol. 2012;80:471-481.
15. Liu B, Zhang FC, Zhang ZL, Zhang W, Gao LX. Interstitial lung disease and Sjögren’s syndrome in primary biliary cirrhosis: A causal or casual association? Clin Rheumatol. 2008;27:1299-1306. doi: 10.1007/ s10067-008-0917-x
16. Franco I, Dubini A, Piciucchi S, Casoni G, Poletti V. Interstitial lung disease preceding primary biliary cirrhosis in a male patient. Rev Port Pneumol. 2015;21:214-217. doi: 10.1016/j.rppnen.2015.02.008
17. Talwalkar JA, Lindor KD. Primary biliary cirrhosis. Lancet. 2003;362:53-61. doi: 10.1016/S0140-6736(03)13808-1
18. Golding PL, Smith M, Williams R. Multisystem involvement in chronic liver disease. Studies on the incidence and pathogenesis. Am J Med. 1973;55:772-782.
19. Strobel ES, Bonnet RB, Werner P, Schaefer HE. Clinical Rheumatology Case Report Bronehiolitis Obliterans Organising Pneumonia and Primary Biliary Cirrhosis-Like Lung Involvement in a Patient with Primary Biliary Cirrhosis. Clin Rheumatol. 1998;17:246-249.
20. Stanley NN, Woodgate DJ. Mottled chest radiograph and gas transfer defect in chronic liver disease. Thorax. 1972;27:315-323.
21. Wallace JG Jr, Tong MJ, Ueki BH, Quismorio FP. Pulmonary involvement in primary biliary cirrhosis. J Clin Gastroenterol. 1987;9(4):
431-435.
22. Chatté G, Streichenberger N, Boillot O, Gille D, Loire R, Cordier J. Lymphocytic bronchitis / bronchiolitis in a patient with primary biliary cirrhosis. Eur Respir J. 1995;8:176-179.
23. Wallwork J, Williams R, Calne RY. Transplantation of liver, heart, and lungs for primary biliary cirrhosis and primary pulmonary hypertension. Lancet. 1987;330:182-185.
24. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Management of cholestatic liver diseases. J Hepatol. 2009;51:237-267. doi: 10.1016/j.jhep.2009.04.009
25. LaRusso NF, Shneider BL, Black D, Gores GJ, James SP, Doo E, Hoofnagle JH. Primary sclerosing cholangitis: Summary of a workshop. Hepatology 2006;44:746-764. doi: 10.1002/hep.21337
26. Jonard P, Geubel A, Wallon J, et al. Primary sclerosing cholangitis and idiopathic pulmonary fibrosis: a case report. Acta Clin. Belg. 1989;44:24-30.
27. Gossard AA, Lindor KD. Autoimmune hepatitis: A review. J Gastroenterol. 2012;47:498-503. doi: 10.1007/s00535-012-0586-z
28. Ferri C, La Civita L, Fazzi P, Solfanelli S, Lombardini F, Begliomini E, Monti M, Longombardo G, Pasero G, Zignego L. Interstitial lung fibrosis and rheumatic disorders in patients with hepatitis C virus infection. Br J Rheumatol. 1997;36:360-365.
29. Мухин Н.А., Абдурахманов Д.Т., Лопаткина Т.Н. и др. Схема Саммерскилла в лечении тяжелых форм аутоиммунного гепатита. Клиническая гепатология. 2011;(3):39-45 [Mukhin NA, Abdurachmanov DT, Lopatkina TN, et al. Summerskill scheme in the treatment of severe forms of autoimmune hepatitis. Klinicheskaya Hepatologia. 2011;(3):39-45 (In Russ.)].

________________________________________________

1. Martusewicz-Boros MM, Boros PW, Wiatr E. Respiratory system involvement in chronic liver diseases. Pol Arch Med Wewn. 2013; 123:635-642. doi: 10.20452/pamw.1980
2. Lv X, Gao F, Liu Q, Zhang S, Huang Z, Zhu Y, Zong H, Li Q, Li S. Clinical and pathological characteristics of IgG4-related interstitial lung disease. Exp Ther Med. 2018;15:1465-1473. doi: 10.3892/etm. 2017.5554
3. Al-Mujaini A, Al-Khabori M, Shenoy K, Wali U. Immunoglobulin
G4-related disease: An update. Oman Med J. 2018;33:97-103.
doi: 10.5001/omj.2018.20
4. Ramachandraiah V, Aronow W, Chandy D. Pulmonary sarcoidosis:
an update. Postgrad Med Taylor & Francis. 2017;129:149-158.
doi: 10.1080/00325481.2017.1251818
5. Mells GF. Primary biliary cirrhosis: Family, genes, and bugs. Clin Liver Dis. 2014;3:69-73. doi: 10.1002/cld.308
6. Shen M, Zhang F, Zhang X. Primary Biliary Cirrhosis Complicated With Interstitial Lung Disease: A Prospective Study in 178 Patients.
J Clin Gastroenterol. 2009;43:676-679. doi: 10.1093/innovait/inq138
7. Koksal D, Koksal AS, Gurakar A. Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis. J Clin Transl Hepatol. 2016;4:258-262. doi: 10.14218/JCTH.2016.00024
8. Rodriguez-Roisin R, Pares A, Bruguera M, Coll J, Picado C. Pulmonary involvement in primary biliary cirrhosis. Thorax. 1981;36: 208-212.
9. Uddenfeldt P, Bjerle P, Danielsson A, Nyström L. Lung Function Abnormalities in Patients with Primary Biliary Cirrhosis. Acta Med Scand. 1988;223:549-555.
10. Krowka MJ, Grambsch PM, Edell ES, Cortese DA, Dickson ER. Primary biliary cirrhosis: relation between hepatic function and pulmonary function in patients who never smoked. Hepatology. 1991;13:1095-1100.
11. Costa C, Sambataro A, Baldi S, Modena V, Todros L, Libertucci D, Coni F, Fusaro E, Revello F, Murgia A. Primary biliary cirrhosis: lung involvement. Liver. 1995;15:196-201.
12. Wallaert B, Bonniere P, Prin L, Cortot A, Tonnel AB, Voisin C. Primary biliary cirrhosis. Subclinical inflammatory alveolitis in patients with normal chest roentgenograms. Chest. 1986;90:842-848.
13. Spiteri MA, Johnson M, Epstein O, Sherlock S, Clarke SW, Poulter LW. Immunological features of lung lavage cells from patients with primary biliary cirrhosis may reflect those seen in pulmonary sarcoidosis. Gut. 1990;31:208-212.
14. Kuś J, Bartosiewicz M, Siemion-Szcześniak I, Jędrych M, Radwan-Röhrenschef P, Lewandowska K, Langfort R, Oniszh K, Franczuk M, Kuś J, Wesołowski S. Interstitial lung disease in patients with primary biliary cirrhosis. Pneumonol Alergol Pol. 2012;80:471-481.
15. Liu B, Zhang FC, Zhang ZL, Zhang W, Gao LX. Interstitial lung disease and Sjögren’s syndrome in primary biliary cirrhosis: A causal or casual association? Clin Rheumatol. 2008;27:1299-1306. doi: 10.1007/ s10067-008-0917-x
16. Franco I, Dubini A, Piciucchi S, Casoni G, Poletti V. Interstitial lung disease preceding primary biliary cirrhosis in a male patient. Rev Port Pneumol. 2015;21:214-217. doi: 10.1016/j.rppnen.2015.02.008
17. Talwalkar JA, Lindor KD. Primary biliary cirrhosis. Lancet. 2003;362:53-61. doi: 10.1016/S0140-6736(03)13808-1
18. Golding PL, Smith M, Williams R. Multisystem involvement in chronic liver disease. Studies on the incidence and pathogenesis. Am J Med. 1973;55:772-782.
19. Strobel ES, Bonnet RB, Werner P, Schaefer HE. Clinical Rheumatology Case Report Bronehiolitis Obliterans Organising Pneumonia and Primary Biliary Cirrhosis-Like Lung Involvement in a Patient with Primary Biliary Cirrhosis. Clin Rheumatol. 1998;17:246-249.
20. Stanley NN, Woodgate DJ. Mottled chest radiograph and gas transfer defect in chronic liver disease. Thorax. 1972;27:315-323.
21. Wallace JG Jr, Tong MJ, Ueki BH, Quismorio FP. Pulmonary involvement in primary biliary cirrhosis. J Clin Gastroenterol. 1987;9(4):
431-435.
22. Chatté G, Streichenberger N, Boillot O, Gille D, Loire R, Cordier J. Lymphocytic bronchitis / bronchiolitis in a patient with primary biliary cirrhosis. Eur Respir J. 1995;8:176-179.
23. Wallwork J, Williams R, Calne RY. Transplantation of liver, heart, and lungs for primary biliary cirrhosis and primary pulmonary hypertension. Lancet. 1987;330:182-185.
24. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Management of cholestatic liver diseases. J Hepatol. 2009;51:237-267. doi: 10.1016/j.jhep.2009.04.009
25. LaRusso NF, Shneider BL, Black D, Gores GJ, James SP, Doo E, Hoofnagle JH. Primary sclerosing cholangitis: Summary of a workshop. Hepatology 2006;44:746-764. doi: 10.1002/hep.21337
26. Jonard P, Geubel A, Wallon J, et al. Primary sclerosing cholangitis and idiopathic pulmonary fibrosis: a case report. Acta Clin. Belg. 1989;44:24-30.
27. Gossard AA, Lindor KD. Autoimmune hepatitis: A review. J Gastroenterol. 2012;47:498-503. doi: 10.1007/s00535-012-0586-z
28. Ferri C, La Civita L, Fazzi P, Solfanelli S, Lombardini F, Begliomini E, Monti M, Longombardo G, Pasero G, Zignego L. Interstitial lung fibrosis and rheumatic disorders in patients with hepatitis C virus infection. Br J Rheumatol. 1997;36:360-365.
29.[Mukhin NA, Abdurachmanov DT, Lopatkina TN, et al. Summerskill scheme in the treatment of severe forms of autoimmune hepatitis. Klinicheskaya Hepatologia. 2011;(3):39-45 (In Russ.)].

Авторы

Л.А. Акулкина 2, М.Ю. Бровко 1, В.И. Шоломова 1,2, Т.П. Розина 1,2, А.Ш. Янакаева 1,
Л.Я. Французевич 2, М.В. Лебедева 1, В.В. Фомин 3

1 Клиника им. Е.М.Тареева ФГБОУ 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;
3 Кафедра факультетской терапии №1 лечебного факультета ФГБОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Москва, Россия

________________________________________________

L.A. Akulkina 2, M.Yu Brovko 1, V.I. Sholomova 1,2, T.P. Rozina 1,2, A.S. Yanakayeva 1, L.Ya. Frantsuzevich 2, M.V. Lebedeva 1, V.V. Fomin 3

1 I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Tareev clinic of Internal Diseases;
2 M.V. Lomonosov Moscow State University, Faculty of Base Medicine, Department of Internal medicine;
3 I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Faculty of Medicine, Chair of Internal Medicine No.1

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