Autoimmune hepatitis classification
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Manpreet Kaur, MD [2]
Overview
According to European Association for the Study of the Liver, There are three types of autoimmune hepatitis based on the types of antibodies present- AIH type 1, AIH type 2, AIH type 3. Overlap syndrome is a type of syndrome found in a patient who presents with the features of AIH, PBC-AIH or PSC-AIH.
Classification
According to European Association for the Study of the Liver, there are three types of autoimmune hepatitis based upon the types of antibodies present:[1][2][3][4][5]
Autoimmune hepatitis Type-1:
- Common type of AIH (accounts almost for 90% of AIH cases) is type one.
- 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 is present.
- 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
Overlap syndrome:
Patients who present with the features of primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC) along with the features of AIH, PBC-AIH or PSC-AIH.[6][7]
| Classification of overlap syndrome | ||
|---|---|---|
| Name of syndrome | Dominant disease | Recessive disease |
| PBC-AIH overlap | PBC | AIH |
| PSC-AIH overlap | PSC | AIH |
| AIH-PBC overlap | AIH | PBC |
| AIH-PSC overlap | AIH | PSC |
| AIH, autoimmune hepatitis; PBC, primary biliary cholangitis; PSC, primary sclerosing cholangitis. | ||
References
- ↑ Sener AG (2015). “Autoantibodies in autoimmune liver diseases”. APMIS. 123 (11): 915–9. doi:10.1111/apm.12442. PMID 26359647.
- ↑ “EASL Clinical Practice Guidelines: Autoimmune hepatitis”. J. Hepatol. 63 (4): 971–1004. 2015. doi:10.1016/j.jhep.2015.06.030. PMID 26341719.
- ↑ 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.
- ↑ 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.
- ↑ Krawitt EL (2006). “Autoimmune hepatitis”. N. Engl. J. Med. 354 (1): 54–66. doi:10.1056/NEJMra050408. PMID 16394302.
- ↑ Kuiper EM, Zondervan PE, van Buuren HR (2010). “Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome”. Clin. Gastroenterol. Hepatol. 8 (6): 530–4. doi:10.1016/j.cgh.2010.03.004. PMID 20304098.
- ↑ van Buuren HR, van Hoogstraten H, Terkivatan T, Schalm SW, Vleggaar FP (2000). “High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis”. J. Hepatol. 33 (4): 543–8. PMID 11059858. Vancouver style error: initials (help)
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