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

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