Autoimmune Hepatitis – Was ist wichtig für die Praxis?

Die autoimmune Hepatitis gehört zu den seltenen immunologisch vermittelten Lebererkrankungen mit chronisch-fluktuierendem Verlauf. Unbehandelt führt sie in den meisten Fällen zur Leberzirrhose mit den damit verbundenen Komplikationen. Dieser Beitrag bietet einen Überblick über klinisch relevante Aspekte bei der Betreuung betroffener Patient/-innen.

Abstract

Autoimmune hepatitis is a rare but chronic autoimmune-mediated liver disease. Key features are elevated transaminases, hypergammaglobulinemia, presence of specific autoantibodies and typical histological features. Diagnostic scores are helpful in establishing the diagnosis. Immunosuppressive therapy should be initiated in every patient with inflammatory activity. First-line therapy includes steroids and azathioprine and results in biochemical and histological remission in the majority of patients. In most cases, lifelong therapy is required. Every patient should receive regular follow-up surveillance including biochemical parameters as well as sonography and elastography.

Schlüsselwörter

autoimmune Hepatitis - autoimmune Lebererkrankungen - Hypergammaglobulinämie - Transaminasenerhöhung - Azathioprin

Key words

autoimmune hepatitis - autoimmune liver diseases - hypergammaglobulinemia - elevated transaminases - azathioprine

Publication History

Article published online:
11 April 2022

• Literatur

• 1 Weiler-Normann C, Lohse AW. Autoimmune hepatitis: from immunopathogenesis to diagnostic and therapeutic innovation. Curr Opin Gastroenterol 2021; 37: 86-90
• 2 European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Autoimmune hepatitis. J Hepatol 2015; 63: 971-1004
• 3 Deutsche Gesellschaft für Gastroenterologie, Verdauungs- und Stoffwechselkrankheiten (DGVS) (federführend). S2k-Leitlinie Autoimmune Lebererkrankungen. Z Gastroenterol 2017; 55: 1135-1226
• 4 Manns MP, Lohse AW, Vergani D. Autoimmune hepatitis – Update 2015. J Hepatol 2015; 62: S100-S111
• 5 Sucher E, Sucher R, Gradistanac T. et al. Autoimmune Hepatitis – Immunologically Triggered Liver Pathogenesis – Diagnostic and Therapeutic Strategies. J Immunol Res 2019; 2019: 9437043
• 6 Muratori P, Fabbri A, Lalanne C. et al. Autoimmune liver disease and concomitant extrahepatic autoimmune disease. Eur J Gastroenterol Hepatol 2015; 27: 1175-1179
• 7 Mack CL, Adams D, Assis DN. et al. 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 2020; 72: 671-722
• 8 Galaski J, Weiler-Normann C, Schakat M. et al. Update of the simplified criteria for autoimmune hepatitis: Evaluation of the methodology for immunoserological testing. J Hepatol 2021; 74: 312-320
• 9 European Association for Study of Liver; Asociacion Latinoamericana para el Estudio del Higado. EASL-ALEH Clinical Practice Guidelines: Non-invasive tests for evaluation of liver disease severity and prognosis. J Hepatol 2015; 63: 237-264
• 10 Hennes EM, Zeniya M, Czaja AJ. et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology 2008; 48: 169-176
• 11 Alvarez F, Berg PA, Bianchi FB. et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 1999; 31: 929-938
• 12 Schmidt T, Schmidt C, Strahl A. et al. A System to Determine Risk of Osteoporosis in Patients With Autoimmune Hepatitis. Clin Gastroenterol Hepatol 2020; 18: 226-233.e3
• 13 Mayer LS, Käser R, Böttler T. Autoimmune cholestatische Lebererkrankungen. Dtsch Med Wochenschr 2020; 145: 296-305
• 14 Hartl J, Ehlken H, Sebode M. et al. Usefulness of biochemical remission and transient elastography in monitoring disease course in autoimmune hepatitis. J Hepatol 2018; 68: 754-763
• 15 Lohse AW, Sebode M, Jørgensen MH. et al. Second-line and third-line therapy for autoimmune hepatitis: A position statement from the European Reference Network on Hepatological Diseases and the International Autoimmune Hepatitis Group. J Hepatol 2020; 73: 1496-1506
• 16 Manns MP, Woynarowski M, Kreisel W. et al. Budesonide induces remission more effectively than prednisone in a controlled trial of patients with autoimmune hepatitis. Gastroenterology 2010; 139: 1198-1206
• 17 Wong LL, Fisher HF, Stocken DD. et al. The Impact of Autoimmune Hepatitis and Its Treatment on Health Utility. Hepatology 2018; 68: 1487-1497
• 18 Pape S, Gevers TJG, Belias M. et al. Predniso(lo)ne Dosage and Chance of Remission in Patients With Autoimmune Hepatitis. Clin Gastroenterol Hepatol 2019; 17: 2068-2075.e2
• 19 van den Brand FF, van der Veen KS, Lissenberg-Witte BI. et al. Adverse events related to low dose corticosteroids in autoimmune hepatitis. Aliment Pharmacol Ther 2019; 50: 1120-1126
• 20 Fachinformation: Azathioprin-ratiopharm® 25 mg/50 mg Filmtabletten. Im Internet (Stand: 14.02.2022): https://www.ratiopharm.de/assets/products/de/label/Azathioprin-ratiopharm%2025%20mg%2050%20mg%20Filmtabletten%20-%202.pdf?pzn=4334324
• 21 Munz M, Pretscher D, Wilhelm M. et al. Azathioprine-induced reversible EBV-associated Hodgkin-like lymphoma after immunosuppressive therapy for autoimmune hepatitis. Int J Clin Pharmacol Ther 2018; 56: 142-147
• 22 Hindorf U, Jahed K, Bergquist A. et al. Characterisation and utility of thiopurine methyltransferase and thiopurine metabolite measurements in autoimmune hepatitis. J Hepatol 2010; 52: 106-111
• 23 Lohse AW, Sebode M, Vesterhus M. Reply to: "Both tacrolimus and mycophenylate mophetil should be considered second-line therapy for autoimmune hepatitis". J Hepatol 2021; 74: 755-756
• 24 Hennes EM, Oo YH, Schramm C. et al. Mycophenolate mofetil as second line therapy in autoimmune hepatitis?. American J Gastroenterol 2008; 103: 3063-3070
• 25 Wahlin S, Efe C. Both tacrolimus and mycophenylate mophetil should be considered second-line therapy for autoimmune hepatitis. J Hepatol 2021; 74: 753-755
• 26 Hübener S, Oo YH, Than NN. et al. Efficacy of 6-Mercaptopurine as Second-Line Treatment for Patients With Autoimmune Hepatitis and Azathioprine Intolerance. Clin Gastroenterol Hepatol 2016; 14: 445-453
• 27 Wagner N, Assmus F, Arendt G. et al. Impfen bei Immundefizienz: Anwendungshinweise zu den von der Ständigen Impfkommission empfohlenen Impfungen. (IV) Impfen bei Autoimmunkrankheiten, bei anderen chronisch-entzündlichen Erkrankungen und unter immunmodulatorischer Therapie. Bundesgesundheitsblatt Gesundheitsforschung Gesundheitsschutz 2019; 62: 494-515
• 28 Boettler T, Marjot T, Newsome PN. et al. Impact of COVID-19 on the care of patients with liver disease: EASL-ESCMID position paper after 6 months of the pandemic. JHEP Rep 2020; 2: 100169
• 29 Zecher B. https://rare-liver.eu/media/covid-19-vaccine_statement_lohse-de.pdf
• 30 Robert Koch-Institut. Empfehlungen der Ständigen Impfkommission. Im Internet (Stand: 14.02.2022): https://www.rki.de/DE/Content/Kommissionen/STIKO/Empfehlungen/Impfempfehlungen_node.html;jsessionid=B3232F401AC132E45BE7815BE3F781D4.internet072
• 31 Marjot T, Buescher G, Sebode M. et al. SARS-CoV-2 infection in patients with autoimmune hepatitis. J Hepatol 2021; 74: 1335-1343
• 32 Fix OK, Hameed B, Fontana RJ. et al. Clinical Best Practice Advice for Hepatology and Liver Transplant Providers During the COVID-19 Pandemic: AASLD Expert Panel Consensus Statement. Hepatology 2020; 72: 287-304
• 33 Bril F, Al Diffalha S, Dean M. et al. Autoimmune hepatitis developing after coronavirus disease 2019 (COVID-19) vaccine: Causality or casualty?. J Hepatol 2021; 75: 222-224
• 34 Kabaçam G, Wahlin S, Efe C. Autoimmune hepatitis triggered by COVID-19: A report of two cases. Liver Int 2021; 41: 2527-2528
• 35 McShane C, Kiat C, Rigby J. et al. The mRNA COVID-19 vaccine – a rare trigger of Autoimmune Hepatitis?. J Hepatol 2021; 75: 1252-1254
• 36 Wissenschaftlicher Dachverband Osteologie (DVO). DVO Leitlinie Osteoporose 2017. Im Internet (Stand: 14.02.2022): https://dv-osteologie.org/osteoporose-leitlinien
• 37 Hoeroldt B, McFarlane E, Dube A. et al. Long-term outcomes of patients with autoimmune hepatitis managed at a nontransplant center. Gastroenterology 2011; 140: 1980-1989