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autoimmune hepatitis

Epidemiology: 1) type 1 a) most common form in USA b) occurs in young women (20-40 years), less often postmenopausal women 2) type 2 a) girls or young women b) a small subset are older patients, including males with concurrent hepatitis C virus infection Pathology: - aggressive, chronic inflammation Clinical manifestations: 1) may be asymptomatic - 1/3 of patients present with acute hepatitis 2) fatigue, jaundice, pruritus common 3) stigmata of cirrhosis - most patients have chronic hepatitis & cirrhosis 4) an associated autoimmune disorder is common (50%) [1] - thyroiditis (hypothyroidism), ulcerative colitis, synovitis Laboratory: 1) abnormal liver function tests a) serum ALT increased (may be very high) [4] b) serum AST increased (may be very high) [4] c) serum bilirubin increased d) elevated or normal serum alkaline phosphatase [4,5,6] 2) anti-smooth muscle antibodies 3) anti-actin autoantibodies (type 1) 4) p-ANCA+ or anti-liver-kidney microsome 1 (anti-LKM1) Ab (type 2) 5) anti-soluble liver antigen (type 3) 6) 4% have false + HCV serology by 2nd generation assay 7) anti-nuclear antibody (ANA) is usually + in high titer (> 1:320) 8) anti RUVBL1 autoantibodies 9) other markers (potential): SEPSECS 10) elevated serum globulins, elevated serum IgG 11) liver biopsy establishes diagnosis [1] 12) see ARUP consult [2] Differential diagnosis: - exclude: - Wilson's disease* - viral hepatitis* - drug-induced liver disease* - Budd-Chiari syndrome* - RUQ pain, hepatomegaly, ascites - primary biliary cholangitis: serum transaminases modestly elevated - hemochromatosis: serum transaminases modestly elevated * may be associated with extreme elevations in serum transaminases Complications: - hepatopulmomary syndrome - platypnea-orthodeoxia syndrome Management: 1) delay treatment until symptomatic [1] 2) type 1 a) corticosteroids as initial therapy (frequent response) b) azathioprine 1] sole therapy 2] in conjunction with corticosteroids (most patients) 3] after initial favorable response to corticosteroids c) duration of therapy 2-3 years prior to consideration of withdrawal [1] - liver biopsy prior to cessation of immunosuppressive therapy [1] - relapse occurs in most patients after cessation of therapy 3) type 2 - often runs an aggressive course with fulminant hepatitis or progression to cirrhosis despite corticosteroids 4) consider liver transplantation for hepatopulmonary syndrome [4]

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autoimmune disease chronic hepatitis cirrhosis

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hepatitis

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2018, 2021. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  2. ARUP Consult: Autoimmune Hepatitis The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/autoimmune-hepatitis
  3. Manns MP, Czaja AJ, Gorham JD, Krawitt EL et al Diagnosis and management of autoimmune hepatitis. Hepatology. 2010 Jun;51(6):2193-213 PMID: 20513004 (corresponding NGC guideline withdrawn Nov 2015)
  4. NEJM Knowledge+ Gastroenterology
  5. Krawitt EL Autoimmune hepatitis N Engl J Med 2006;354:54-66. PMID: 16394302 https://www.nejm.org/doi/pdf/10.1056/NEJMra050408
  6. 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 Aug;72(2):671-722. PMID: 31863477 No abstract available.
  7. Autoimmune Hepatitis https://www.niddk.nih.gov/health-information/liver-disease/autoimmune-hepatitis