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Autoimmune Liver Disease - Kuwait
- 1. Autoimmune Liver Diseases: AIH, PBC, PSC Neil Theise, MD Depts. of Pathology and Medicine (Digestive Diseases) Beth Israel Medical Center – Albert Einstein College of Medicine New York City
- 3. Gender: Mostly female Autoimmune Hepatitis:
- 4. Gender: Mostly female Age: 40’s - 60’s Autoimmune Hepatitis:
- 5. Gender: Mostly female Age: 40’s - 60’s Autoantibodies: anti-nuclear (ANA) anti-smooth muscle (ASMA) anti-mitochondrial (AMA) anti-liver kidney microsomal 1 (LKM1) Autoimmune Hepatitis:
- 6. Gender: Mostly female Age: 40’s - 60’s Autoantibodies: anti-nuclear (ANA) anti-smooth muscle (ASMA) anti-mitochondrial (AMA) anti-liver kidney microsomal 1 (LKM1) Presentation: Very severe activity &/or very late stage Autoimmune Hepatitis:
- 7. Dynamics of Activity and Progression of Autoimmune Hepatitis over Time Assessment is NOT like chronic viral hepatitis!
- 8. Stage of Disease Time Viral hepatitis: Progression vs. activity over time
- 9. Stage of Disease Time Autoimmune hepatitis: Progression vs. activity over time
- 10. Stage of Disease Biopsy: Advanced disease, but mild or “burned out hepatitis” Autoimmune hepatitis: Progression vs. activity over time
- 11. Biopsy: Advancing disease and severe activity Stage of Disease Autoimmune hepatitis: Progression vs. activity over time
- 12. Biopsy: Little scarring, but severe activity Stage of Disease Autoimmune hepatitis: Progression vs. activity over time
- 13. Stage of Disease Time Autoimmune hepatitis: Progression vs. activity over time No biopsy is likely to show: mild activity and little scarring…
- 14. Gender: Mostly female Age: 40’s - 60’s Autoantibodies: anti-nuclear (ANA) anti-smooth muscle (ASMA) anti-mitochondrial (AMA) anti-liver kidney microsomal 1 (LKM1) Presentation: Very severe activity &/or very late stage Treatment: Immune suppression, transplant for endstage. Autoimmune Hepatitis:
- 15. Autoimmune Hepatitis: plasma cells
- 17. Gender: Women Primary Biliary Cirrhosis (PBC):
- 18. Gender: Women Age: Middle aged to older Primary Biliary Cirrhosis (PBC):
- 19. Gender: Women Age: Middle aged to older Associated diseases: Scleroderma Hashimoto’s thyroiditis CREST syndrome Sjogren’s syndrome, etc…. Primary Biliary Cirrhosis (PBC):
- 20. Gender: Women Age: Middle aged to older Associated diseases: Scleroderma Hashimoto’s thyroiditis CREST syndrome Sjogren’s syndrome, etc…. Serologic markers: AMA (ASMA, ANA) Primary Biliary Cirrhosis (PBC):
- 21. Gender: Women Age: Middle aged to older Associated diseases: Scleroderma Hashimoto’s thyroiditis CREST syndrome Sjogren’s syndrome, etc…. Serologic markers: AMA (ASMA, ANA) Bile ducts involved: Medium to small ducts ONLY Primary Biliary Cirrhosis (PBC):
- 22. PBC, Stage 1: Bile duct injury/loss
- 26. PBC, Stage 2: Ductular reaction
- 29. PBC, Stage 3: Fibrosis From portal fibrosis to Transition to cirrhosis…
- 30. PBC, Stage 4: Cirrhosis
- 34. PBC AIH
- 35. PBC AIH
- 36. PBC AIH “ Overlap” Hepatitis
- 37. PBC AIH “ Overlap” Syndrome
- 38. PBC AIH NRH
- 39. Gender: Men Primary Sclerosing Cholangitis (PSC):
- 40. Gender: Men Age: Teens to 30’s Primary Sclerosing Cholangitis (PSC):
- 41. Gender: Men Age: Teens to 30’s Associated diseases: Inflammatory bowel disease ( U.C. or Crohn’s) 70% have IBD 70% of IBD will develop PSC Primary Sclerosing Cholangitis (PSC):
- 42. Gender: Men Age: Teens to 30’s Associated diseases: Inflammatory bowel disease ( U.C. or Crohn’s) 70% have IBD 70% of IBD will develop PSC Serologic markers: ANCA…? Primary Sclerosing Cholangitis (PSC):
- 43. Gender: Men Age: Teens to 30’s Associated diseases: Inflammatory bowel disease ( U.C. or Crohn’s) 70% have IBD 70% of IBD will develop PSC Serologic markers: ANCA…? Ducts involved: Large extrahepatic to small intrahepatic Primary Sclerosing Cholangitis (PSC):
- 44. Gender: Men Age: Teens to 30’s Associated diseases: Inflammatory bowel disease ( U.C. or Crohn’s) 70% have IBD 70% of IBD will develop PSC Serologic markers: ANCA…? Ducts involved: Large extrahepatic to small intrahepatic At increased risk for: Cholangiocarcinoma Primary Sclerosing Cholangitis (PSC):
- 60. Tombstone Scar
- 61. Tombstone Scars
- 62. Tombstone Scars
- 64. In situ High Grade Dysplasia
- 65. In situ papillary cholangiocarcinoma
- 69. Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) vs. Age Gender Assoc’d Dx 40-60 Female RA, CREST Scleroderma Sjogren’s 10-30 Male Ulcerative Colitis Crohn’s Disease
- 70. Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) vs. Age Gender Assoc’d Dx 40-60 Female RA, CREST Scleroderma, Sjogren’s 10-30 Male Ulcerative Colitis Crohn’s Disease
- 71. Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) vs. Age Gender Assoc’d Dx 40-60 Female RA, CREST Scleroderma, Sjogren’s 10-30 Male Ulcerative Colitis Crohn’s Disease
- 72. Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) vs. Age Gender Assoc’d Dx Ducts Affected 40-60 Female RA, CREST Scleroderma, Sjogren’s Small to medium 10-30 Male Ulcerative Colitis Crohn’s Disease All ducts
- 73. Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) vs. Age Gender Assoc’d Dx Ducts Affected Method of Dx 40-60 Female RA, CREST Scleroderma, Sjogren’s Small to medium Biopsy 10-30 Male Ulcerative Colitis Crohn’s Disease All ducts MRCP/ERCP
- 74. vs. P S C S UC KS P B C B UCKS NOT Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) MNEMONIC!!!
- 75. vs. P S C S UC KS P B C B UCKS NOT Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) MNEMONIC!!!
Notes de l'éditeur
- Fig. 16.1A. Variation in the severity of fibrosis in chronic biliary disease. These two slides are taken from a single block of liver obtained at transplantation from a patient with primary biliary cirrhosis. One area shows established cirrhosis (A). In an adjacent area there is preservation of normal vascular relationships with no evidence of fibrosis (B). (P = portal tract, H = hepatic venule). H&E.
- Fig. 16.1B. Variation in the severity of fibrosis in chronic biliary disease. These two slides are taken from a single block of liver obtained at transplantation from a patient with primary biliary cirrhosis. One area shows established cirrhosis (A). In an adjacent area there is preservation of normal vascular relationships with no evidence of fibrosis (B). (P = portal tract, H = hepatic venule). H&E.
- Fig. 12.7B. Metaplasia of gallbladder mucosa in chronic cholecystitis. (A) A lobule of pyloric glands is seen in the lamina propria. The surface epithelium is of gastric foveolar type. (B) Intestinal metaplasia with columnar and goblet cells; endocrine cells were identified by immunohistochemistry. H&E.
- Fig. 11.8B. (A) Suppurative cholangitis with cholangitic abscess (*) and remnants of the bile-duct epithelium (arrow) in a case of bacterial cholangitis. (B) Non-suppurative cholangitis showing epithelial damage (arrow) and periductal infiltration of lymphocytes and plasma cells in primary biliary cirrhosis. H & E.
- Fig. 11.7B. Biliary epithelial dysplasia associated with hepatolithiasis. (A) Lining epithelium of a large intrahepatic bile duct shows micropapillary hyperplasia with cellular dysplasia. H & E. (B) Close-up of micropapillary hyperplasia with mild dysplasia illustrating nuclear pseudostratification. H & E.
- Fig. 15.62. Intrahepatic cholangiocarcinoma. This peripheral tumour has replaced the entire left lobe.
- Fig. 15.63. Intrahepatic cholangiocarcinoma. This lobectomy specimen contains a hard, yellow-white tumour that arose near the hilum and spread peripherally.
- Fig. 15.65. Cholangiocarcinoma. Poorly formed glands with frequent mitoses. H&E.