autoimmune hepatitis
Jump to navigation
Jump to search
Epidemiology
- type 1
- most common form in USA
- occurs in young women (20-40 years), less often postmenopausal women
- type 2
- girls or young women
- a small subset are older patients, including males with concurrent hepatitis C virus infection
Pathology
- aggressive, chronic inflammation
Clinical manifestations
- may be asymptomatic
- 1/3 of patients present with acute hepatitis
- fatigue, jaundice, pruritus common
- stigmata of cirrhosis
- most patients have chronic hepatitis & cirrhosis
- an associated autoimmune disorder is common (50%)[1]
Laboratory
- abnormal liver function tests
- serum ALT increased (may be very high)[4]
- serum AST increased (may be very high)[4]
- serum bilirubin increased
- elevated or normal serum alkaline phosphatase[4][5][6]
- anti-smooth muscle antibodies
- anti-actin autoantibodies (type 1)
- p-ANCA+ or anti-liver-kidney microsome 1 (anti-LKM1) Ab (type 2)
- anti-soluble liver antigen (type 3)
- 4% have false + HCV serology by 2nd generation assay
- anti-nuclear antibody (ANA) is usually + in high titer (> 1:320)
- anti RUVBL1 autoantibodies
- other markers (potential): SEPSECS
- elevated serum globulins, elevated serum IgG
- liver biopsy establishes diagnosis[1]
- see ARUP consult[2]
Complications
- hepatopulmomary syndrome
Differential diagnosis
- exclude:
- Wilson's disease*
- viral hepatitis*
- drug-induced liver disease*
- Budd-Chiari syndrome*
- primary biliary cholangitis: serum transaminases modestly elevated
- hemochromatosis: serum transaminases modestly elevated
* may be associated with extreme elevations in serum transaminases
Management
- delay treatment until symptomatic[1]
- type 1
- corticosteroids as initial therapy (frequent response)
- azathioprine
- sole therapy
- in conjunction with corticosteroids (most patients)
- after initial favorable response to corticosteroids
- 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
- type 2
- often runs an aggressive course with fulminant hepatitis or progression to cirrhosis despite corticosteroids
- consider liver transplantation for hepatopulmonary syndrome[4]
More general terms
Additional terms
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 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 - ↑ 2.0 2.1 ARUP Consult: Autoimmune Hepatitis The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/autoimmune-hepatitis
- ↑ Manns MP, Czaja AJ, Gorham JD, Krawitt EL et al Diagnosis and management of autoimmune hepatitis. Hepatology. 2010 Jun;51(6):2193-213 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20513004 (corresponding NGC guideline withdrawn Nov 2015)
- ↑ 4.0 4.1 4.2 4.3 4.4 NEJM Knowledge+ Gastroenterology
- ↑ 5.0 5.1 Krawitt EL Autoimmune hepatitis N Engl J Med 2006;354:54-66. PMID: https://www.ncbi.nlm.nih.gov/pubmed/16394302 https://www.nejm.org/doi/pdf/10.1056/NEJMra050408
- ↑ 6.0 6.1 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: https://www.ncbi.nlm.nih.gov/pubmed/31863477 No abstract available.
- ↑ Autoimmune Hepatitis https://www.niddk.nih.gov/health-information/liver-disease/autoimmune-hepatitis