Autoimmune hepatitis classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: :Manpreet Kaur, MD [2]

Overview

Classification

According to European Association for the Study of the Liver, there are three types of Autoimmune hepatitis according to types of antibodies present:[1][2][3][4][5]

Autoimmune hepatitis Type-1:

  • Common type of AIH (accounts almost for 90% of AIH cases)
  • Presents at any age and prevalent worldwide
  • Antibodies like ANA(antinuclear antibodies), SMA(smooth muscle antibodies) or anti-SLA/LP(soluble liver antigen/liver pancreas antibodies) are characteristics
  • Antiactin antibodies are more specific
  • Association with HLA(human leukocyte antigens)DR3, DR4, and DR13
  • Treatment failure is rare but if relapse occurs after withdrawal of treatment and long-term maintenance therapy is given occasionally

Autoimmune hepatitis Type 2:

  • Accounts for 10% of AIH cases
  • Presents usually in childhood and young adulthood and prevalent worldwide but rare in North America
  • Antibodies like anti-LKM1(liver/kidney microsomal antibody type 1), anti-LC1(antibodies against liver cytosol type 1 antigen) and rarely anti-LKM3(liver/kidney microsomal antibody type 3) are characteristics
  • Anti–LKM-1 and anti–LC-1 are more specific
  • Association with HLA DR3 and DR7
  • Treatment failure is common and frequent relapse rates after withdrawal of treatment and long-term maintenance therapy commonly is given

Autoimmune hepatitis Type 3:

  • AIH type 3 is similar to AIH type 1 but presents more severe.
  • SLA/LP(soluble liver antigen/liver pancreas antibodies) is characteristics
  • Ro52-antibody positive

References

  1. Sener AG (2015). "Autoantibodies in autoimmune liver diseases". APMIS. 123 (11): 915–9. doi:10.1111/apm.12442. PMID 26359647.
  2. "EASL Clinical Practice Guidelines: Autoimmune hepatitis". J. Hepatol. 63 (4): 971–1004. 2015. doi:10.1016/j.jhep.2015.06.030. PMID 26341719.
  3. Floreani A, Niro G, Rosa Rizzotto E, Antoniazzi S, Ferrara F, Carderi I, Baldo V, Premoli A, Olivero F, Morello E, Durazzo M (2006). "Type I autoimmune hepatitis: clinical course and outcome in an Italian multicentre study". Aliment. Pharmacol. Ther. 24 (7): 1051–7. doi:10.1111/j.1365-2036.2006.03104.x. PMID 16984499.
  4. Liberal R, Mieli-Vergani G, Vergani D (2013). "Clinical significance of autoantibodies in autoimmune hepatitis". J. Autoimmun. 46: 17–24. doi:10.1016/j.jaut.2013.08.001. PMID 24016388.
  5. Krawitt EL (2006). "Autoimmune hepatitis". N. Engl. J. Med. 354 (1): 54–66. doi:10.1056/NEJMra050408. PMID 16394302.

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