Autoimmune hepatitis classification: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Autoimmune hepatitis}} | {{Autoimmune hepatitis}} | ||
{{CMG}}; {{AE}} | {{CMG}}; {{AE}}{{MKK}} | ||
==Overview== | ==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. | 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== | ==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''':<ref name="pmid26359647">{{cite journal |vauthors=Sener AG |title=Autoantibodies in autoimmune liver diseases |journal=APMIS |volume=123 |issue=11 |pages=915–9 |year=2015 |pmid=26359647 |doi=10.1111/apm.12442 |url=}}</ref><ref name="pmid26341719">{{cite journal |vauthors= |title=EASL Clinical Practice Guidelines: Autoimmune hepatitis |journal=J. Hepatol. |volume=63 |issue=4 |pages=971–1004 |year=2015 |pmid=26341719 |doi=10.1016/j.jhep.2015.06.030 |url=}}</ref><ref name="pmid16984499">{{cite journal |vauthors=Floreani A, Niro G, Rosa Rizzotto E, Antoniazzi S, Ferrara F, Carderi I, Baldo V, Premoli A, Olivero F, Morello E, Durazzo M |title=Type I autoimmune hepatitis: clinical course and outcome in an Italian multicentre study |journal=Aliment. Pharmacol. Ther. |volume=24 |issue=7 |pages=1051–7 |year=2006 |pmid=16984499 |doi=10.1111/j.1365-2036.2006.03104.x |url=}}</ref><ref name="pmid24016388">{{cite journal |vauthors=Liberal R, Mieli-Vergani G, Vergani D |title=Clinical significance of autoantibodies in autoimmune hepatitis |journal=J. Autoimmun. |volume=46 |issue= |pages=17–24 |year=2013 |pmid=24016388 |doi=10.1016/j.jaut.2013.08.001 |url=}}</ref><ref name="pmid16394302">{{cite journal |vauthors=Krawitt EL |title=Autoimmune hepatitis |journal=N. Engl. J. Med. |volume=354 |issue=1 |pages=54–66 |year=2006 |pmid=16394302 |doi=10.1056/NEJMra050408 |url=}}</ref> | '''According to European Association for the Study of the Liver, there are three types of Autoimmune hepatitis based upon the types of antibodies present''':<ref name="pmid26359647">{{cite journal |vauthors=Sener AG |title=Autoantibodies in autoimmune liver diseases |journal=APMIS |volume=123 |issue=11 |pages=915–9 |year=2015 |pmid=26359647 |doi=10.1111/apm.12442 |url=}}</ref><ref name="pmid26341719">{{cite journal |vauthors= |title=EASL Clinical Practice Guidelines: Autoimmune hepatitis |journal=J. Hepatol. |volume=63 |issue=4 |pages=971–1004 |year=2015 |pmid=26341719 |doi=10.1016/j.jhep.2015.06.030 |url=}}</ref><ref name="pmid16984499">{{cite journal |vauthors=Floreani A, Niro G, Rosa Rizzotto E, Antoniazzi S, Ferrara F, Carderi I, Baldo V, Premoli A, Olivero F, Morello E, Durazzo M |title=Type I autoimmune hepatitis: clinical course and outcome in an Italian multicentre study |journal=Aliment. Pharmacol. Ther. |volume=24 |issue=7 |pages=1051–7 |year=2006 |pmid=16984499 |doi=10.1111/j.1365-2036.2006.03104.x |url=}}</ref><ref name="pmid24016388">{{cite journal |vauthors=Liberal R, Mieli-Vergani G, Vergani D |title=Clinical significance of autoantibodies in autoimmune hepatitis |journal=J. Autoimmun. |volume=46 |issue= |pages=17–24 |year=2013 |pmid=24016388 |doi=10.1016/j.jaut.2013.08.001 |url=}}</ref><ref name="pmid16394302">{{cite journal |vauthors=Krawitt EL |title=Autoimmune hepatitis |journal=N. Engl. J. Med. |volume=354 |issue=1 |pages=54–66 |year=2006 |pmid=16394302 |doi=10.1056/NEJMra050408 |url=}}</ref> | ||
'''Autoimmune hepatitis Type-1''': | '''Autoimmune hepatitis Type-1''': | ||
*Common type of AIH (accounts almost for 90% of AIH cases) | *Common type of AIH (accounts almost for 90% of AIH cases) is type one. | ||
*Presents at any age and prevalent worldwide | *Presents at any age and prevalent worldwide. | ||
*Antibodies like [[ANA]]([[antinuclear antibodies]]), [[SMA]](smooth muscle antibodies) or anti- | *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 | *Antiactin [[antibodies]] are more specific. | ||
*Association with HLA([[human leukocyte antigens]])DR3, DR4, and DR13 | *Association with HLA ([[human leukocyte antigens]]) [[HLA-DR3|DR3]], [[HLA-DR4|DR4]], and [[HLA-DR13|DR13]] is present. | ||
*Treatment failure is rare but if relapse occurs after withdrawal of treatment and long-term maintenance therapy is given occasionally | *Treatment failure is rare but if relapse occurs after withdrawal of treatment and long-term maintenance therapy is given occasionally. | ||
'''Autoimmune hepatitis Type 2''': | '''Autoimmune hepatitis Type 2''': | ||
*Accounts for 10% of AIH cases | *Accounts for 10% of AIH cases | ||
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*Anti–LKM-1 and anti–LC-1 are more specific | *Anti–LKM-1 and anti–LC-1 are more specific | ||
*Association with [[HLA DR3-DQ2|HLA DR3]] and DR7 | *Association with [[HLA DR3-DQ2|HLA DR3]] and DR7 | ||
*Treatment failure is common and frequent relapse rates after | *Treatment failure is common and frequent relapse rates after withdrawal of treatment, and long-term maintenance therapy commonly is given. | ||
'''Autoimmune hepatitis Type 3''': | '''Autoimmune hepatitis Type 3''': | ||
*AIH type 3 is similar to AIH type 1 but presents more severe. | *AIH type 3 is similar to AIH type 1 but presents more severe. | ||
*SLA/LP(soluble liver antigen/liver pancreas antibodies) is characteristics | *SLA/LP (soluble liver antigen/liver pancreas antibodies) is characteristics. | ||
*Ro52-[[antibody]] positive | *Ro52-[[antibody]] positive | ||
'''Overlap syndrome''': | '''Overlap syndrome''': | ||
Patients who present with the features of | Patients who present with the features of [[primary biliary cirrhosis]] ([[PBC]]) or [[primary sclerosing cholangitis]] ([[Primary sclerosing cholangitis|PSC]]) along with the features of AIH, [[PBC]]-AIH or [[PSC]]-AIH.<ref name="pmid20304098">{{cite journal |vauthors=Kuiper EM, Zondervan PE, van Buuren HR |title=Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome |journal=Clin. Gastroenterol. Hepatol. |volume=8 |issue=6 |pages=530–4 |year=2010 |pmid=20304098 |doi=10.1016/j.cgh.2010.03.004 |url=}}</ref><ref name="pmid11059858">{{cite journal |vauthors=van Buuren HR, van Hoogstraten HJE, Terkivatan T, Schalm SW, Vleggaar FP |title=High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis |journal=J. Hepatol. |volume=33 |issue=4 |pages=543–8 |year=2000 |pmid=11059858 |doi= |url=}}</ref> | ||
{| class="wikitable" | {| class="wikitable" | ||
! colspan="3" |Classification of overlap syndrome | ! colspan="3" |Classification of overlap syndrome | ||
Revision as of 15:13, 9 January 2018
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Autoimmune hepatitis Microchapters |
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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
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)