PRIMARY SCLEROSING CHOLANGITIS

PRIMARY SCLEROSING CHOLANGITIS


PRIMARY SCLEROSING CHOLANGITIS

  • PSC is characterised by development of multiple strictures and dilation of CBD with features of fibrous thickening of CBD.

 

ETIOLOGY-

  • Associated with ulcerative colitis (MC), crohn’s disease, (Inflammatory bowel disease), grave’s disease, Sjogren’s syndrome

 

CLINICAL FEATURES-

  • Associated with elevation of gamma- glutanyl transpeptidase initially
  • anatomic site of origin, as intrahepatic, hilar (central, 65% of CCCs), and peripheral (or distal, 30% of CCCs).
  • Formation of multiple strictures
  • Present in young adults
  • Jaundice
  • Increased risk of cholangiocarcinoma
  • Malabsorption (especially of fat) and steatorrhea, leading to decreased levels of the fat-soluble vitamins, A, D, E and K.
  • Signs of cirrhosis
  • The disease progresses to progressive biliary fibrous stricturing.
  •  The concentric periductal fibrosis around the affected ducts (“onion skin fibrosis”) is followed by their disappearance leaving behind a solid cord-like fibrous scar
  • Usual presentation is intermittent jaundice, fatigue, weight loss, pruritus and abdominal pain. 

 

INVESTIGATIONS-

  1. Ultrasound- intra and extrahepatic dilations
  2. MRCP- non invasive investigations shows multiple strictures and dilation
  3. ERCP- IOC for strictures in the CBD and dilation has “beaded appearance”
  4. Increased alkaline phosphatase

 

TREATMENT-

  • Liver transplantation- TOC
  • For temporary relief- by biliary stent

Exam Important

ETIOLOGY-

  • Associated with ulcerative colitis (MC), crohn’s disease, (Inflammatory bowel disease), grave’s disease, Sjogren’s syndrome

 

CLINICAL FEATURES-

  • Associated with elevation of gamma- glutanyl transpeptidase initially
  • anatomic site of origin, as intrahepatic, hilar (central, 65% of CCCs), and peripheral (or distal, 30% of CCCs).
  • Formation of multiple strictures
  • Present in young adults
  • Jaundice
  • Increased risk of cholangiocarcinoma
  • Malabsorption (especially of fat) and steatorrhea, leading to decreased levels of the fat-soluble vitamins, A, D, E and K.
  • Signs of cirrhosis
  • The disease progresses to progressive biliary fibrous stricturing.
  •  The concentric periductal fibrosis around the affected ducts (“onion skin fibrosis”) is followed by their disappearance leaving behind a solid cord-like fibrous scar
  • Usual presentation is intermittent jaundice, fatigue, weight loss, pruritus and abdominal pain. 

 

INVESTIGATIONS-

  1. Ultrasound- intra and extrahepatic dilations
  2. MRCP- non invasive investigations shows multiple strictures and dilation
  3. ERCP- IOC for strictures in the CBD and dilation has “beaded appearance”
  4. Increased alkaline phosphatase

 

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