PSC mainly affects males and is characterized by chronic inflammation of the bile ducts resulting in stricture formation and obliteration of the duct lumen. In 70% of the patients it is associated with inflammatory bowel disease (IBD).
Presentation and diagnosis:
Jaundice and bacterial cholangitis is a common mode of presentation. Presence of cholangitis indicates imaging of the biliary tree for locating dominant stricture that may require dilatation. Presence of recurrent episodes of cholangitis is in itself an indicator to refer the patient for liver transplantation even if the synthetic function of the liver is normal. Pruritus is a common presentation. This may be present with normal bilurubin level and in the absence of dominant stricture. Hepatic osteodystrophy, a condition resulting in gross reduction in bone mineral content is common and may lead to fracture in long bone or the spine. 70% of the patients have IBD and colonoscopy is indicated to rule out colonic dysplasia or cancer. Cholangiocarcinoma may complicate PSC and usually results in sudden increase in jaundice, weight loss and serum alkaline phosphatase level. The diagnosis of PSC is primarily by liver biopsy.
The Mayo clinic model uses age, histology staging, bilurubin level and absence of splenomegaly to prgnosticate outcome. The King's college model uses age, histological grade, hepatomegaly, splenomegaly and serum alkaline phosphatase level. However these are not very useful in clinical situations. Other factors such as nutritional staus, cholangitis and risk of cholangio carcinoma dictate the outcome.
Identification of dominant strictures in the biliary tree needs dilatation and stent placement. Episodes of recurrent cholangitis (biliary tree infection) requires suppressive dose of antibiotics. H1 receptor blockade may alleviate pruritus (itching). Majority of the patients will require liver replacement fairly early. Unlike other chronic liver diseases the indication for listing for liver transplantation is for recurrent cholangitis, progressive jaundice and nutritional debility than poor synthetic function of the liver.