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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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 immunologic feature that appear to favor an overall “

1950 : association of AIH and ANAs, lupoid hepatitis

10 – 20% of all cases of chronic hepatitis


Immunodiagnosis immunologic feature that appear to favor an overall “

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.


Immunologic tests immunologic feature that appear to favor an overall “


Typical immunologic feature that appear to favor an overall “ immunoserologic Profiles in Autoimmune Diseases of Liver


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

MSKL

RSKL


Lkm1 antibodies

LKM1 Antibodies : stomach, kidney, liver combined tissue blocks

The Dx criteria of international AIH group

The use of IFA titer , Not ELISA

IFA performed in early questionable cases


Lkm2 antibodies
LKM2 ANTIBODIES: stomach, kidney, liver combined tissue blocks

Demonstrated in some drug induced hepatitis

No indication since the drugs was withdrawn


Lkm3 antibodies
LKM3 stomach, kidney, liver combined tissue blocks ANTIBODIES :

Detectable in < 20% of HDV , HCV viral infections

For differentiation of virus induced AIH


Useful Tests stomach, kidney, liver combined tissue blocks in Autoimmune Liver Diseases


Microsomal stomach, kidney, liver combined tissue blocks Autoantibodies in Autoimmune Liver Disease


Typical stomach, kidney, liver combined tissue blocks Antimitochondrial and Liver-Kidney-Microsomal Autoantibody Patterns With Indirect Immunofluorescence


Smooth Muscle stomach, kidney, liver combined tissue blocks Autoantibodies, SMA

Heterogenous groups of auto Ab that target specific cytoskeleton filaments

Actin

Vimentin

Desmins

Tubulins


Specific auto stomach, kidney, liver combined tissue blocks 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


Methods stomach, kidney, liver combined tissue blocks

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


Test interpretation stomach, kidney, liver combined tissue blocks

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


Simplified Scoring system stomach, kidney, liver combined tissue blocks


Other related autoantibodies
Other Related stomach, kidney, liver combined tissue blocks Autoantibodies

  • SLA/LP

  • LC1

  • LKM2, LKM3

  • LM

  • ASGPR


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