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Autoimmune Hepatitis

فهرست

Autoimmune hepatitis (AIH) is a chronic and inflammatory liver disease with unknown etiology. AIH can trigger as acute hepatitis and go forward to chronic liver disease and cirrhosis. Clinical symptoms and biochemistry tests are very different in acute hepatitis and cirrhosis.
Autoimmune hepatitis is identified two types:
Type 1: it’s more common type and the first onset occur at all age. This type is more related to other autoimmune disorders like celiac disease, rheumatoid arthritis and ulcerative colitis.
Type 2: it’s happened in children and young individuals.


Panel Test:

● Liver function testing:
  o Transaminases (AST and ALT)

● Hepatitis testing: important to rule out acute or chronic hepatitis
  o Hepatitis A virus [HAV]
  o Hepatitis B virus [HBV]
  o Hepatitis C virus [HCV])

● Quantitative immunoglobulins:
  o IgG usually elevated

● Autoantibodies:
  o Antinuclear antibody (ANA)
  o Antineutrophil cytoplasmic antibody (ANCA)
  o Antismooth muscle antibody (anti-SMA)
  o Anti-F-actin (smooth muscle) antibody
  o Antimitochondrial antibody (AMA)
  o Antisoluble liver antigen antibody (anti-SLA)
  o Antiliver-kidney microsomal-1 antibody (anti-LKM-1; cytochrome P450 2D6)
  o Antiliver cytosol (anti-LC-1)

Comment: Serum titers of antibodies do not appear to correlate with disease activity. Titers may vary during course of disease without activity correlation.

● Immunogenetics:
  o Association with HLA-B1, B8, DR3 and DR4

References

Guidelines
1. Mieli-Vergani G, Vergani D, Baumann U, Czubkowski P, Debray D, Dezsofi A, Fischler B, Gupte G, Hierro L, Indolfi G, Jahnel J, Smets F, Verkade HJ, Hadzic N. Diagnosis and Management of Pediatric Autoimmune Liver Disease: ESPGHAN Hepatology Committee Position Statement. J Pediatr Gastroenterol Nutr. 2018; 66(2): 345-360. PubMed
2. Manns MP, Czaja AJ, Gorham JD, Krawitt EL, Mieli-Vergani G, Vergani D, Vierling JM, American Association for the Study of Liver Diseases. Diagnosis and management of autoimmune hepatitis Hepatology. 2010; 51(6): 2193-213. PubMed
3. International Autoimmune Hepatitis Group. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology. 2008; 48(1): 169-76. PubMed
4. Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, Gores GJ, American Association for the Study of Liver Diseases. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010; 51(2): 660-78. PubMed
5. Gleeson D, Heneghan MA, British society of Gastroenterology. British Society of Gastroenterology (BSG) guidelines for management of autoimmune hepatitis. Gut. 2011; 60(12): 1611-29. PubMed
6. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Autoimmune hepatitis. J Hepatol. 2015; 63(4): 971-1004. PubMed

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