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{{Autoimmune hepatitis}}
{{Autoimmune hepatitis}}
{{CMG}}; {{AE}}:{{MKK}}
{{CMG}}; {{AE}}{{MKK}}
==Overview==
==Overview==
[[Liver biopsy]] is the gold standard test for the diagnosis of [[autoimmune hepatitis]]. Interface [[hepatitis]] (ie, piecemeal [[necrosis]]) is the histological hallmark of [[autoimmune hepatitis]]. In [[cirrhosis]], [[fibrosis]], bridging [[necrosis]] are usually found. Liver biospy is also used to diagnose overlap syndrome.
== Diagnostic Study of Choice ==
== Diagnostic Study of Choice ==
=== Gold standard/Study of choice: ===
*[[Liver biopsy]] is the gold standard test for the diagnosis of [[autoimmune hepatitis]]<ref name="pmid12362575">{{cite journal |vauthors=Carpenter HA, Czaja AJ |title=The role of histologic evaluation in the diagnosis and management of autoimmune hepatitis and its variants |journal=Clin Liver Dis |volume=6 |issue=3 |pages=685–705 |year=2002 |pmid=12362575 |doi= |url=}}</ref><ref name="pmid17051746">{{cite journal |vauthors=Szabó Z |title=[The role of liver biopsy in the diagnosis of autoimmune hepatitis] |language=Hungarian |journal=Orv Hetil |volume=147 |issue=35 |pages=1697–702 |year=2006 |pmid=17051746 |doi= |url=}}</ref><ref name="pmid21674554">{{cite journal |vauthors=Suzuki A, Brunt EM, Kleiner DE, Miquel R, Smyrk TC, Andrade RJ, Lucena MI, Castiella A, Lindor K, Björnsson E |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 |year=2011 |pmid=21674554 |pmc=3192933 |doi=10.1002/hep.24481 |url=}}</ref><ref name="pmid26642062">{{cite journal |vauthors=Tiniakos DG, Brain JG, Bury YA |title=Role of Histopathology in Autoimmune Hepatitis |journal=Dig Dis |volume=33 Suppl 2 |issue= |pages=53–64 |year=2015 |pmid=26642062 |doi=10.1159/000440747 |url=}}</ref>
*Liver biopsy is the gold standard test for the diagnosis of Autoimmune hepatitis
*Liver biopsy is performed by following methods:
*Liver biopsy is performed by following methods:
**Percutaneous liver biopsy with or without ultrasound guidance
**Percutaneous liver biopsy with or without [[ultrasound]] guidance
**Transjugular liver biopsy
**Transjugular liver biopsy
Histology findings usually found on biopsy:
Histology findings usually found on biopsy:
*Interface hepatitis (ie, piecemeal necrosis) is the histological hallmark
*Interface [[hepatitis]] (ie, piecemeal [[necrosis]]) is the histological hallmark.
**There is lymphoplasmacytic periportal infiltrate which invade the limiting plate
**Portal lesion spares the [[biliary tree]]
**There is an abundance of plasma cells and eosinophils are usually present
**This involves most of the [[lobule]]
**Portal lesion spares the biliary tree
**Lobular collapse which is best identified by reticulin staining.
*A plasma cell infiltrate lead to plasma cell hepatitis, lobular, or panacinar hepatitis
**There is lymphoplasmacytic periportal infiltrate which invade the limiting plate.
*Bridging necrosis
**There is an abundance of [[plasma cells]] and [[eosinophils]] are usually present.
*Fibrosis  
*A plasma cell infiltrate lead to plasma cell [[hepatitis]], If it involves lobules called lobular or whole liver known as panacinar [[hepatitis]].
*Fibrosis is present in all but the mildest forms of AIH. It causes distortion of the hepatic lobule and the appearance of regenerative nodules, resulting in cirrhosis
In [[cirrhosis]] following findings are seen on biopsy:
*[[Fibrosis]]
*Bridging [[necrosis]]


==Overlap syndrome==
Liver biospy is also used to diagnose overlap syndrome:<ref name="pmid20101749">{{cite journal |vauthors=Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, Gores GJ |title=Diagnosis and management of primary sclerosing cholangitis |journal=Hepatology |volume=51 |issue=2 |pages=660–78 |year=2010 |pmid=20101749 |doi=10.1002/hep.23294 |url=}}</ref><ref name="pmid12122860">{{cite journal |vauthors=Dienes HP, Erberich H, Dries V, Schirmacher P, Lohse A |title=Autoimmune hepatitis and overlap syndromes |journal=Clin Liver Dis |volume=6 |issue=2 |pages=349–62, vi |year=2002 |pmid=12122860 |doi= |url=}}</ref>
{| class="wikitable"
! colspan="2" | '''Histology findings of overlap syndrome'''
|-
|Types
|Biopsy findings
|-
|AIH-PBC
|
* Interface [[hepatitis]]
* Destructive [[cholangitis]] (florid duct lesions) 
|-
|AIH-PSC
|
* Interface [[hepatitis]]


Fibrosis is present in most patients with autoimmune hepatitis. Without effective therapy, fibrosis starts to connect the portal and central areas, which ultimately leads to cirrhosis.
* Ductopenia
 
* Portal edema or fibrous
 
* Obliterative fibrous [[cholangitis]] (rare)
|-
|AIH-cholestatic syndrome
|
* Interface [[hepatitis]]
 
* Destructive [[cholangitis]] or bile duct loss
|}


==References==
==References==

Latest revision as of 17:27, 9 January 2018

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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

Liver biopsy is the gold standard test for the diagnosis of autoimmune hepatitis. Interface hepatitis (ie, piecemeal necrosis) is the histological hallmark of autoimmune hepatitis. In cirrhosis, fibrosis, bridging necrosis are usually found. Liver biospy is also used to diagnose overlap syndrome.

Diagnostic Study of Choice

Histology findings usually found on biopsy:

  • Interface hepatitis (ie, piecemeal necrosis) is the histological hallmark.
    • Portal lesion spares the biliary tree
    • This involves most of the lobule
    • Lobular collapse which is best identified by reticulin staining.
    • There is lymphoplasmacytic periportal infiltrate which invade the limiting plate.
    • There is an abundance of plasma cells and eosinophils are usually present.
  • A plasma cell infiltrate lead to plasma cell hepatitis, If it involves lobules called lobular or whole liver known as panacinar hepatitis.

In cirrhosis following findings are seen on biopsy:

Overlap syndrome

Liver biospy is also used to diagnose overlap syndrome:[5][6]

Histology findings of overlap syndrome
Types Biopsy findings
AIH-PBC
AIH-PSC
  • Ductopenia
  • Portal edema or fibrous
AIH-cholestatic syndrome

References

  1. Carpenter HA, Czaja AJ (2002). "The role of histologic evaluation in the diagnosis and management of autoimmune hepatitis and its variants". Clin Liver Dis. 6 (3): 685–705. PMID 12362575.
  2. Szabó Z (2006). "[The role of liver biopsy in the diagnosis of autoimmune hepatitis]". Orv Hetil (in Hungarian). 147 (35): 1697–702. PMID 17051746.
  3. Suzuki A, Brunt EM, Kleiner DE, Miquel R, Smyrk TC, Andrade RJ, Lucena MI, Castiella A, Lindor K, Björnsson E (2011). "The use of liver biopsy evaluation in discrimination of idiopathic autoimmune hepatitis versus drug-induced liver injury". Hepatology. 54 (3): 931–9. doi:10.1002/hep.24481. PMC 3192933. PMID 21674554.
  4. Tiniakos DG, Brain JG, Bury YA (2015). "Role of Histopathology in Autoimmune Hepatitis". Dig Dis. 33 Suppl 2: 53–64. doi:10.1159/000440747. PMID 26642062.
  5. Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, Gores GJ (2010). "Diagnosis and management of primary sclerosing cholangitis". Hepatology. 51 (2): 660–78. doi:10.1002/hep.23294. PMID 20101749.
  6. Dienes HP, Erberich H, Dries V, Schirmacher P, Lohse A (2002). "Autoimmune hepatitis and overlap syndromes". Clin Liver Dis. 6 (2): 349–62, vi. PMID 12122860.

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