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Biliary Tract Disorders

Intra Hepatic Biliary Disease

    1. Etiology: Extra hepatic bile duct obstruction: stones, atresia, stricture, carcinoma Q
    2. Conjugated bil. , S. Alkaline PO4ase Bile acids cholesterol
    3. C/F Pruritis, jaundice, dark urine, light stools
    4. M/C Bile stasis with bile duct proliferation
    5. Jigsaw pattern due to coarse fibrous septae Small and large bile ducts in septae 
  2. Primary Biliary CIRRHOSIS –
    1. Female: Male = 6:1, middle age (40-50 yrs)
    2. Chronic, progressive, often fatal cholestatic liver disease
    3. Destruction of intra hepatic bile ducts - Medium sized ducts
    4. Serum Auto antibodies: Anti mitochondrial Ab (in 90%) Q against E2 subunit of pyruvate dehydrogenase complex of (IgM type).       
    5. Extra hepatic manifestation - Sjögren’s syndrome, scleroderma, Thyroiditis, Membranous glomerulonephritis, celiac disease, Raynaud’s phenomenon. Q 
    6. Morphology:
      Focal and variable disease
      (Diff. Degrees of severity in different portions of liver)
    7. Stages:
  • Florid duct lesion (by granulomatous inflammation) - Destruction of terminal and conducting bile ducts.
  • Ductular proliferation
  • Scarring
  • Cirrhosis
    Bile stained green liver
  1. Primary Sclerosing Cholangitis: Q -
    1. Inflammation and obliterative fibrosis of intra and extrahepatic bile ducts, with dilatation of preserved segments
    2. Male > Female 3-5th decade
    3. Obliterative, fibrosing cholangitis (onion skin)
    4. Segmental obliteration of intra and extra hepatic bile duct
    5. Beading of Barium column
    6. Associated with IBD Q (ulcerative colitis in 70%)
    7. risk of cholangiocarcinoma Q
    8. Treatment: Liver transplantation
Differences between PBC & PSC


Primary Biliary Cirrhosis

Primary Sclerosing Cholangitis


Median age 50 years (30-70)

Median age 30 years


90% female

70% male

Clinical course


Unpredictable but progressive

Associated conditions

Sjögren syndrome (70%)

Inflammatory bowel disease (70%)


Scleroderma (5%)

Pancreatitis (≤25%)


Thyroid disease (20%)

Idiopathic fibrosing diseases (retroperitoneal fibrosis)


95% AMA positive

0% to 5% AMA positive (low titer)


20% ANA positive

6% ANA positive


60% ANCA positive

82% ANCA positive



Strictures and beading of large bile ducts; pruning of smaller ducts

Duct lesion

Florid duct lesion; loss of small ducts

Concentric periductal fibrosis; loss of small ducts

Anomalies of Biliary Tree

  1. Von Meyenburg complex / Bile duct micro hamartomas Q
    Incomplete involution of embryonic bile duct remnants
    1. Within PT: small clusters of dilated bile ducts embedded in fibrous, stromaQ
    2. May communicate with biliary tree
  2. Polycystic Liver Disease
    1. Multiple diffuse cystic lesions Q
    2. No pigmented material. Detached from biliary tree
  3. Congenital Hepatic Fibrosis
    1. PT enlarged by irregular Broad bands of collagen Q
    2. Divide liver into irregular Islands
    3. Variable no. of abnormal, shaped bile ducts embedded in fibrous tissue
      a. In continuity with biliary tree
      b. May dev. PHT, ↑ risk of cholangio Carcinoma
  4. Caroli's Disease:
    1. Larger ducts of intra hepatic biliary tree are segmentally dilated and contain inspissated bile Q
    2. Feature: Intra hepatic cholelithiasis, Cholangitis
    3. ­ Risk of cholangiocarcinoma, Portal H. T
  5. Alagille syndrome :
  1. Paucity of bile ducts Q
  2. Un common AD condition
  3. Liver (n) .but PT bile ducts absent
  1. Peculiar facies, vertebral abnorm, CVS defects Q
  2. Risk of hepatic failure & HCC
  3. Mutations in gene Jagged 1 on chromosome 20p: encodes ligands for notch 1
  4. (Role in development of organ systems).

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