Autoimmune diseases of the liver
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Autoimmune Diseases of The Liver . As an anatomic site, the liver exhibit distinctive immunologic feature that appear to favor an overall “ tolerogenic microenvironment”. Autoimmune Hepatitis. 1950 : association of AIH and ANAs, lupoid hepatitis

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Autoimmune Diseases of The Liver

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Autoimmune diseases of the liver

Autoimmune Diseases of The Liver


Autoimmune diseases of the liver

As an anatomic site, the liver exhibit distinctive immunologic feature that appear to favor an overall “ tolerogenic microenvironment”


1950 association of aih and anas lupoid hepatitis 10 20 of all cases of chronic hepatitis

Autoimmune Hepatitis

1950 : association of AIH and ANAs, lupoid hepatitis

10 – 20% of all cases of chronic hepatitis


Autoimmune diseases of the liver

Immunodiagnosis

for the purpose clinical management, patient present with chronic liver inflammation

Differential Dx : chronic viral hepatitis, drug induced hepatitis , fatty liver diseases

Clinical lab characterize autoimmune liver disease by identifying distinct auto Ab.


Autoimmune diseases of the liver

Immunologic tests


Autoimmune diseases of the liver

Typical immunoserologic Profiles in Autoimmune Diseases of Liver


Autoimmune diseases of the liver

LKM, SMA , AMAs can be identified using mouse or rat stomach, kidney, liver combined tissue blocks

MSKL

RSKL


Lkm1 antibodies

LKM1 Antibodies :

The Dx criteria of international AIH group

The use of IFA titer , Not ELISA

IFA performed in early questionable cases


Lkm2 antibodies

LKM2 ANTIBODIES:

Demonstrated in some drug induced hepatitis

No indication since the drugs was withdrawn


Lkm3 antibodies

LKM3 ANTIBODIES :

Detectable in < 20% of HDV , HCV viral infections

For differentiation of virus induced AIH


Autoimmune diseases of the liver

Useful Tests in Autoimmune Liver Diseases


Autoimmune diseases of the liver

MicrosomalAutoantibodies in Autoimmune Liver Disease


Autoimmune diseases of the liver

Typical Antimitochondrial and Liver-Kidney-Microsomal Autoantibody Patterns With Indirect Immunofluorescence


Autoimmune diseases of the liver

Smooth MuscleAutoantibodies, SMA

Heterogenous groups of auto Ab that target specific cytoskeleton filaments

Actin

Vimentin

Desmins

Tubulins


Autoimmune diseases of the liver

Specific auto Ab to F-actin (antiactin) are a SMA subset characteristic of AIH

F-actin is the biologically active polymeric form of actin found and functioning in muscle contraction and cellular processes


Autoimmune diseases of the liver

Methods

IFA using unfixed frozen section of RSKL , MSKL as substrate

Homogenous fluorescence of stomach muscularis mucosa and intramucosal muscles and mesangial cells and media of blood vessels


Autoimmune diseases of the liver

Test interpretation

SMA sensitive but not specific for AIH

SMA often coexist with ANAs

> 1/80 identified in AIH, but lower titer in PSC, PBC

> 1/320 correlate with anti-actin specificity

< 5% in normal individuals


Autoimmune diseases of the liver

Simplified Scoring system


Other related autoantibodies

Other Related Autoantibodies

  • SLA/LP

  • LC1

  • LKM2, LKM3

  • LM

  • ASGPR


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