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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”. Autoimmune Hepatitis. 1950 : association of AIH and ANAs, lupoid hepatitis

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Autoimmune Diseases of The Liver

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  1. Autoimmune Diseases of The Liver

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

  3. Autoimmune Hepatitis 1950 : association of AIH and ANAs, lupoid hepatitis 10 – 20% of all cases of chronic hepatitis

  4. 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.

  5. Immunologic tests

  6. Typical immunoserologic Profiles in Autoimmune Diseases of Liver

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

  8. LKM1 Antibodies : The Dx criteria of international AIH group The use of IFA titer , Not ELISA IFA performed in early questionable cases

  9. LKM2 ANTIBODIES: Demonstrated in some drug induced hepatitis No indication since the drugs was withdrawn

  10. LKM3 ANTIBODIES : Detectable in < 20% of HDV , HCV viral infections For differentiation of virus induced AIH

  11. Useful Tests in Autoimmune Liver Diseases

  12. MicrosomalAutoantibodies in Autoimmune Liver Disease

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

  14. Smooth MuscleAutoantibodies, SMA Heterogenous groups of auto Ab that target specific cytoskeleton filaments Actin Vimentin Desmins Tubulins

  15. 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

  16. 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

  17. 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

  18. Simplified Scoring system

  19. Other Related Autoantibodies • SLA/LP • LC1 • LKM2, LKM3 • LM • ASGPR

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