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Autoimmune Liver Diseases: AIH, PBC, PSC Neil Theise, MD Depts. of Pathology and Medicine (Digestive Diseases) Beth Israel...
Autoimmune Hepatitis:
Gender: Mostly female Autoimmune Hepatitis:
Gender: Mostly female Age: 40’s - 60’s Autoimmune Hepatitis:
Gender: Mostly female Age: 40’s - 60’s Autoantibodies: anti-nuclear (ANA) anti-smooth muscle (ASMA) anti-mitochondrial (AM...
Gender: Mostly female Age: 40’s - 60’s Autoantibodies: anti-nuclear (ANA) anti-smooth muscle (ASMA) anti-mitochondrial (AM...
Dynamics of Activity and Progression of Autoimmune Hepatitis over Time Assessment is NOT like chronic viral hepatitis!
Stage of Disease Time Viral hepatitis: Progression vs. activity over time
Stage of Disease Time Autoimmune hepatitis: Progression vs. activity over time
Stage of Disease Biopsy: Advanced disease, but mild or “burned out hepatitis” Autoimmune hepatitis: Progression vs. activi...
Biopsy: Advancing disease and severe activity Stage of Disease Autoimmune hepatitis: Progression vs. activity over time
Biopsy: Little scarring, but severe activity Stage of Disease Autoimmune hepatitis: Progression vs. activity over time
Stage of Disease Time Autoimmune hepatitis: Progression vs. activity over time No biopsy is likely to show: mild activity ...
Gender: Mostly female Age: 40’s - 60’s Autoantibodies: anti-nuclear (ANA) anti-smooth muscle (ASMA) anti-mitochondrial (AM...
Autoimmune Hepatitis: plasma cells
Perivenular confluent necrosis
Gender: Women Primary Biliary Cirrhosis (PBC):
Gender: Women Age: Middle aged to older Primary Biliary Cirrhosis (PBC):
Gender: Women Age: Middle aged to older Associated diseases:  Scleroderma Hashimoto’s thyroiditis CREST syndrome Sjogren’s...
Gender: Women Age: Middle aged to older Associated diseases:  Scleroderma Hashimoto’s thyroiditis CREST syndrome Sjogren’s...
Gender: Women Age: Middle aged to older Associated diseases:  Scleroderma Hashimoto’s thyroiditis CREST syndrome Sjogren’s...
PBC, Stage 1: Bile duct injury/loss
 
 
 
PBC, Stage 2: Ductular reaction
 
 
PBC, Stage 3: Fibrosis From portal fibrosis to Transition to cirrhosis…
PBC, Stage 4: Cirrhosis
<ul><li>PBC, Endstage: </li></ul><ul><li>Clinically, most often: </li></ul><ul><li>Severe cholestatic disease </li></ul><u...
 
 
PBC AIH
PBC AIH
PBC AIH “ Overlap” Hepatitis
PBC AIH “ Overlap” Syndrome
PBC AIH NRH
Gender: Men Primary Sclerosing Cholangitis (PSC):
Gender: Men Age: Teens to 30’s Primary Sclerosing Cholangitis (PSC):
Gender: Men Age: Teens to 30’s Associated diseases:  Inflammatory bowel disease ( U.C. or Crohn’s) 70% have IBD 70% of IBD...
Gender: Men Age: Teens to 30’s Associated diseases:  Inflammatory bowel disease ( U.C. or Crohn’s) 70% have IBD 70% of IBD...
Gender: Men Age: Teens to 30’s Associated diseases:  Inflammatory bowel disease ( U.C. or Crohn’s) 70% have IBD 70% of IBD...
Gender: Men Age: Teens to 30’s Associated diseases:  Inflammatory bowel disease ( U.C. or Crohn’s) 70% have IBD 70% of IBD...
Histology of PSC <ul><li>Extrahepatic pancreato-biliary duct changes  </li></ul><ul><li>Chronic inflammation </li></ul><ul...
Histology of PSC <ul><li>Extrahepatic pancreato-biliary duct changes  </li></ul><ul><li>Chronic inflammation </li></ul><ul...
 
 
 
 
Histology of PSC <ul><li>Extrahepatic pancreato-biliary duct changes  </li></ul><ul><li>Chronic inflammation </li></ul><ul...
 
 
 
 
 
 
 
 
Tombstone Scar
Tombstone Scars
Tombstone Scars
Histology of PSC <ul><li>Extrahepatic pancreato-biliary duct changes  </li></ul><ul><li>Chronic inflammation </li></ul><ul...
In situ High Grade Dysplasia
In situ papillary cholangiocarcinoma
Cholangiocarcinoma
 
 
Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) vs. Age Gender Assoc’d Dx 40-60 Female RA, CREST Scle...
Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) vs. Age Gender Assoc’d Dx 40-60 Female RA, CREST Scle...
Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) vs. Age Gender Assoc’d Dx 40-60 Female RA, CREST Scle...
Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) vs. Age Gender Assoc’d Dx Ducts Affected 40-60 Female...
Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) vs. Age Gender Assoc’d Dx Ducts Affected Method of Dx...
vs. P S C  S  UC KS P B C  B UCKS NOT Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) MNEMONIC!!!
vs. P S C  S  UC KS P B C  B UCKS NOT Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) MNEMONIC!!!
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Autoimmune Liver Disease - Kuwait

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  • 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&amp;amp;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&amp;amp;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&amp;amp;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 &amp;amp; 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 &amp;amp; E. (B) Close-up of micropapillary hyperplasia with mild dysplasia illustrating nuclear pseudostratification. H &amp;amp; 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&amp;amp;E.
  • Transcript of "Autoimmune Liver Disease - Kuwait"

    1. 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
    2. 2. Autoimmune Hepatitis:
    3. 3. Gender: Mostly female Autoimmune Hepatitis:
    4. 4. Gender: Mostly female Age: 40’s - 60’s Autoimmune Hepatitis:
    5. 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. 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. 7. Dynamics of Activity and Progression of Autoimmune Hepatitis over Time Assessment is NOT like chronic viral hepatitis!
    8. 8. Stage of Disease Time Viral hepatitis: Progression vs. activity over time
    9. 9. Stage of Disease Time Autoimmune hepatitis: Progression vs. activity over time
    10. 10. Stage of Disease Biopsy: Advanced disease, but mild or “burned out hepatitis” Autoimmune hepatitis: Progression vs. activity over time
    11. 11. Biopsy: Advancing disease and severe activity Stage of Disease Autoimmune hepatitis: Progression vs. activity over time
    12. 12. Biopsy: Little scarring, but severe activity Stage of Disease Autoimmune hepatitis: Progression vs. activity over time
    13. 13. Stage of Disease Time Autoimmune hepatitis: Progression vs. activity over time No biopsy is likely to show: mild activity and little scarring…
    14. 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. 15. Autoimmune Hepatitis: plasma cells
    16. 16. Perivenular confluent necrosis
    17. 17. Gender: Women Primary Biliary Cirrhosis (PBC):
    18. 18. Gender: Women Age: Middle aged to older Primary Biliary Cirrhosis (PBC):
    19. 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. 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. 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. 22. PBC, Stage 1: Bile duct injury/loss
    23. 26. PBC, Stage 2: Ductular reaction
    24. 29. PBC, Stage 3: Fibrosis From portal fibrosis to Transition to cirrhosis…
    25. 30. PBC, Stage 4: Cirrhosis
    26. 31. <ul><li>PBC, Endstage: </li></ul><ul><li>Clinically, most often: </li></ul><ul><li>Severe cholestatic disease </li></ul><ul><li>or </li></ul><ul><li>Portal hypertension (ascites, varisces, etc.) </li></ul><ul><li>Livers are usually not fully cirrhotic! </li></ul><ul><li>… stage 3 to 4… </li></ul>
    27. 34. PBC AIH
    28. 35. PBC AIH
    29. 36. PBC AIH “ Overlap” Hepatitis
    30. 37. PBC AIH “ Overlap” Syndrome
    31. 38. PBC AIH NRH
    32. 39. Gender: Men Primary Sclerosing Cholangitis (PSC):
    33. 40. Gender: Men Age: Teens to 30’s Primary Sclerosing Cholangitis (PSC):
    34. 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):
    35. 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):
    36. 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):
    37. 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):
    38. 45. Histology of PSC <ul><li>Extrahepatic pancreato-biliary duct changes </li></ul><ul><li>Chronic inflammation </li></ul><ul><li>Focal ulceration </li></ul><ul><li>Strictures: inflammation/edema >>> scar </li></ul>
    39. 46. Histology of PSC <ul><li>Extrahepatic pancreato-biliary duct changes </li></ul><ul><li>Chronic inflammation </li></ul><ul><li>Focal ulceration </li></ul><ul><li>Strictures: inflammation/edema >>> scar </li></ul>> Looks like Ulcerative Colitis! Histology of PSC
    40. 51. Histology of PSC <ul><li>Extrahepatic pancreato-biliary duct changes </li></ul><ul><li>Chronic inflammation </li></ul><ul><li>Focal ulceration </li></ul><ul><li>Strictures: inflammation/edema >>> scar </li></ul><ul><li>Intrahepatic bile duct changes </li></ul><ul><li>Atrophy </li></ul><ul><li>Circumferential, “onion skin” fibrosis </li></ul><ul><li>“ Tombstone scars” </li></ul><ul><li>+/- mild mononuclear portal infiltrates </li></ul>> Looks like Ulcerative Colitis! Histology of PSC
    41. 60. Tombstone Scar
    42. 61. Tombstone Scars
    43. 62. Tombstone Scars
    44. 63. Histology of PSC <ul><li>Extrahepatic pancreato-biliary duct changes </li></ul><ul><li>Chronic inflammation </li></ul><ul><li>Focal ulceration </li></ul><ul><li>Strictures: inflammation/edema >>> scar </li></ul><ul><li>Intrahepatic bile duct changes </li></ul><ul><li>Atrophy </li></ul><ul><li>Circumferential, “onion skin” fibrosis </li></ul><ul><li>“ Tombstone scars” </li></ul><ul><li>+/- mild mononuclear portal infiltrates </li></ul>> Looks like Ulcerative Colitis! <ul><li>Biliary neoplasia </li></ul><ul><li>Progressive dysplasia </li></ul><ul><li>Severe dysplasia = carcinoma in situ </li></ul><ul><li>Cholangiocarcinoma </li></ul>Histology of PSC
    45. 64. In situ High Grade Dysplasia
    46. 65. In situ papillary cholangiocarcinoma
    47. 66. Cholangiocarcinoma
    48. 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
    49. 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
    50. 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
    51. 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
    52. 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
    53. 74. vs. P S C S UC KS P B C B UCKS NOT Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) MNEMONIC!!!
    54. 75. vs. P S C S UC KS P B C B UCKS NOT Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis (PSC) MNEMONIC!!!
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