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Difference between revisions of "Autoimmune hepatitis"
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Autoimmune hepatitis (view source)
Revision as of 21:01, 30 September 2021
, 21:01, 30 September 2021PBC -> change to newer term
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| Micro = interface hepatitis with plasma cells, +/- bile duct injury | | Micro = interface hepatitis with plasma cells, +/- bile duct injury | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = [[viral hepatitis]], [[primary biliary | | LMDDx = [[viral hepatitis]], [[primary biliary cholangitis]], AIH-[[PBC]] overlap, [[PSC]]-AIH overlap, [[drug reaction]] | ||
| Stains = | | Stains = | ||
| IHC = | | IHC = | ||
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| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = | ||
| Clinicalhx = | | Clinicalhx = usu. women - young or middle-aged | ||
| Signs = | | Signs = | ||
| Symptoms = | | Symptoms = | ||
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| Rads = | | Rads = | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = | | Prognosis = poor without treatment | ||
| Other = | | Other = | ||
| ClinDDx = [[primary biliary | | ClinDDx = [[primary biliary cholangitis]], [[primary sclerosing cholangitis]] | ||
| Tx = corticosteroids, immunosuppression, liver transplant (end stage) | |||
}} | }} | ||
'''Autoimmune hepatitis''', abbreviated '''AIH''', in an uncommon [[medical liver disease]] that occurs in adults and children. | '''Autoimmune hepatitis''', abbreviated '''AIH''', in an uncommon [[medical liver disease]] that occurs in adults and children. | ||
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==General== | ==General== | ||
*Several criteria exist to diagnose and histology (alone) is '''not''' sufficient. | *Several criteria exist to diagnose and histology (alone) is '''not''' sufficient. | ||
*Classically, young and middle aged women.<ref name=pmid24367228>{{Cite journal | last1 = Francque | first1 = S. | last2 = Vonghia | first2 = L. | last3 = Ramon | first3 = A. | last4 = Michielsen | first4 = P. | title = Epidemiology and treatment of autoimmune hepatitis. | journal = Hepat Med | volume = 4 | issue = | pages = 1-10 | month = | year = 2012 | doi = 10.2147/HMER.S16321 | PMID = 24367228 }}</ref> | |||
**May occur in men. | |||
*Often a fluctuating course. | |||
*Untreated AIH has a poor outcome.<ref name=pmid24367228/> | |||
**Leads to [[cirrhosis]] and liver failure. | |||
===Diagnosis=== | ===Diagnosis=== | ||
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===Treatment=== | ===Treatment=== | ||
*Immunosuppresants (prednisone, azathioprine).<ref name=pmid19452572>Non-classical phenotypes of autoimmune hepatitis and advances in diagnosis and treatment. Czaja AJ, Bayraktar Y. World J Gastroenterol. 2009 May 21;15(19):2314-28. Review. PMID 19452572.</ref> | *Immunosuppresants (prednisone, azathioprine).<ref name=pmid19452572>Non-classical phenotypes of autoimmune hepatitis and advances in diagnosis and treatment. Czaja AJ, Bayraktar Y. World J Gastroenterol. 2009 May 21;15(19):2314-28. Review. PMID 19452572.</ref> | ||
*Liver transplant (end-stage disease).<ref name=pmid24367228/> | |||
==Microscopic== | ==Microscopic== | ||
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***Lymphoplasmacytic infiltration of portal tracts / lobule. | ***Lymphoplasmacytic infiltration of portal tracts / lobule. | ||
****Periportal [[plasma cells]] - '''key feature'''.<ref>{{Ref PCPBoD8|448}}</ref> | ****Periportal [[plasma cells]] - '''key feature'''.<ref>{{Ref PCPBoD8|448}}</ref> | ||
**Emperipolesis - one cell penetrates into another one (uncommon finding). | **[[Emperipolesis]] - one cell penetrates into another one (uncommon finding). | ||
**Hepatic rosette - inflammatory cells around reactive hepatocytes.<ref name=pmid20527613>{{Cite journal | last1 = Malik | first1 = TA. | last2 = Saeed | first2 = S. | title = Autoimmune hepatitis: a review. | journal = J Pak Med Assoc | volume = 60 | issue = 5 | pages = 381-7 | month = May | year = 2010 | doi = | PMID = 20527613 | url=http://www.jpma.org.pk/full_article_text.php?article_id=2051 }}</ref> | **Hepatic rosette - inflammatory cells around reactive hepatocytes.<ref name=pmid20527613>{{Cite journal | last1 = Malik | first1 = TA. | last2 = Saeed | first2 = S. | title = Autoimmune hepatitis: a review. | journal = J Pak Med Assoc | volume = 60 | issue = 5 | pages = 381-7 | month = May | year = 2010 | doi = | PMID = 20527613 | url=http://www.jpma.org.pk/full_article_text.php?article_id=2051 }}</ref> | ||
*Compatible: | *Compatible: | ||
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**Lots of plasma cells should prompt consideration of AIH. | **Lots of plasma cells should prompt consideration of AIH. | ||
*Atypical Autoimmune hepatitis may have zone III involvment (lymphoplasmacytic infiltrate)<ref name=pmid19452572>Non-classical phenotypes of autoimmune hepatitis and advances in diagnosis and treatment. Czaja AJ, Bayraktar Y. World J Gastroenterol. 2009 May 21;15(19):2314-28. Review. PMID 19452572.</ref> and a normal IgG.<ref>FW. 21 September 2009.</ref> | *Atypical Autoimmune hepatitis may have zone III involvment (lymphoplasmacytic infiltrate)<ref name=pmid19452572>Non-classical phenotypes of autoimmune hepatitis and advances in diagnosis and treatment. Czaja AJ, Bayraktar Y. World J Gastroenterol. 2009 May 21;15(19):2314-28. Review. PMID 19452572.</ref> and a normal IgG.<ref>FW. 21 September 2009.</ref> | ||
DDx: | |||
*[[Viral hepatitis]] - should have plasma cells. | |||
*[[Drug-induced liver injury]] - milder changes than AIH, portal [[neutrophil]]s and [[cholestasis]].<ref>{{Cite journal | last1 = Suzuki | first1 = A. | last2 = Brunt | first2 = EM. | last3 = Kleiner | first3 = DE. | last4 = Miquel | first4 = R. | last5 = Smyrk | first5 = TC. | last6 = Andrade | first6 = RJ. | last7 = Lucena | first7 = MI. | last8 = Castiella | first8 = A. | last9 = Lindor | first9 = K. | title = The use of liver biopsy evaluation in discrimination of idiopathic autoimmune hepatitis versus drug-induced liver injury. | journal = Hepatology | volume = 54 | issue = 3 | pages = 931-9 | month = Sep | year = 2011 | doi = 10.1002/hep.24481 | PMID = 21674554 }}</ref> | |||
*[[Primary biliary cholangitis]]. | |||
*AIH-PBC overlap. | |||
*PSC-AIH overlap. | |||
===Images=== | ===Images=== | ||
Table show [[AIH-nonCIR]] | |||
{| | |||
[[File:1 AIH 2 680x512px.tif|Expanded portal regions with dull edges suggestive of interface hepatitis (40X).]] | |||
[[File:2 AIH 2 680x512px.tif|PAS without diastase shows interface hepatitis (200X).]] | |||
[[File:3 AIH 2 680x512px.tif|Abundant plasma cells seen at higher power (400X).]] | |||
[[File:4 AIH 2 680x512px.tif|PAS with diastase shows intense inflammation of portal triad stroma, with some reduplication of ductal epithelium. The patient’s vial serology and anti-microbial antibody were negative. (200X).]] | |||
|} | |||
Autoimmune hepatitis. Expanded portal regions with dull edges suggestive of interface hepatitis (UL 40X). PAS without diastase shows interface hepatitis (UR 200X). Abundant plasma cells seen at higher power (LL 400X). PAS with diastase shows intense inflammation of portal triad stroma, with some reduplication of ductal epithelium. The patient’s vial serology and anti-microbial antibody were negative. (LR 200X). | |||
Table show [[AIH-CIR]] | |||
{| | |||
[[File:CIrrhosis Mult Cause - 1 - 400X 1369x1024px shot 04.tif|Trichrome shows blue fibrosis about hepatocyte nodules]] | |||
[[File:CIrrhosis Mult Cause - 1 - 400X 1369x1024px shot 01.tif|Inflammation includes occasional plasma cells (yellow arrowhead)]] | |||
[[File:CIrrhosis Mult Cause - 1 - 400X 1369x1024px shot 02.tif|Ballooning degeneration is present]] | |||
[[File:CIrrhosis Mult Cause - 1 - 400X 1369x1024px shot 03.tif|PAS without diastase shows piecemeal necrosis (not very good on wedge biopsies in my experience)]] | |||
|} | |||
Chronic hepatitis with changes of autoimmune hepatitis and with cirrhosis and steatosis. Trichrome shows blue fibrosis about hepatocyte nodules with steatosis (UL, 40X). Inflammation includes occasional plasma cells (UR, 400X, yellow arrowhead at plasma cell), consistent with history of autoimmune hepatitis. Ballooning degeneration is present (LL, 400X). PAS without diastase shows piecemeal necrosis, (LR, 400X). The patient had also had diabetes. | |||
<gallery> | <gallery> | ||
Image:Autoimmune_hepatitis_-_low_mag.jpg | AIH - low mag. (WC) | Image:Autoimmune_hepatitis_-_low_mag.jpg | AIH - low mag. (WC) | ||
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serology is suggested, if not done. | serology is suggested, if not done. | ||
The histology is not suggestive of primary biliary | The histology is not suggestive of primary biliary cholangitis. | ||
</pre> | </pre> | ||
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[[Category:Liver pathology]] | [[Category:Liver pathology]] | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Medical liver disease]] | |||