Autoimmune Hepatitis

From Anvita Health Wiki

Contents 1 Epidemiology 2 Pathology 3 Clinical-manifestations 4 Laboratory 5 Management 6 More General Terms 7 Additional Terms 8 References

Epidemiology

• type 1

• most common form in USA
• occurs in young women, 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

• 1/3 of patients present with acute hepatitis
• stigmata of cirrhosis

• most patients have chronic hepatitis & cirrhosis

• an associated autoimmune disorder is common (i.e. hypothyroidism, etc)

Laboratory

• abnormal liver function tests

• serum ALT increased
• serum AST increased
• serum bilirubin increased
• low to normal serum alkaline phosphatase

• anti-smooth muscle antibodies
• anti-actin autoantibodies (type 1)
• anti-liver- kidney microsome 1 ( anti-LKM1) antibodies (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, serum IgG
• liver biopsy

Management

• delay treatment until symptomatic [1]
• type 1

• corticosteroids as initial therapy (frequent response)
• azathioprine

• sole therapy
• in conjunction with corticosteroids
• after initial favorable response to corticosteroids

• type 2

• often runs an aggressive course with fulminant hepatitis or progression to cirrhosis despite corticosteroids

More General Terms

• hepatitis

Additional Terms

• chronic hepatitis
• cirrhosis

References

1. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15 American College of Physicians, Philadelphia 1998, 2006, 2009
2. Autoimmune Hepatitis [1]
3. National Guideline Clearinghouse Diagnosis and management of autoimmune hepatitis. ngc-guideline: [2]

autoimmune hepatitis