Primary Sclerosing Cholangitis (PSC)



Primary Sclerosing Cholangitis (PSC)





DEFINITION:



  • Idiopathic cholestasis with fibrosis, stricturing and dilation of intra- and extrahepatic bile ducts (small and large bile ducts)


EPIDEMIOLOGY:



  • Young men (age 20-50)


  • May be autoimmune-linked: Associated IBD (U/C more common than CD) in 70% of cases usually appears before PSC; If not get colonoscopy at PSC diagnosis


  • Some believe there may be a viral connection with CMV, REO (respiratory enteric orphan virus)


ETIOLOGIES:



  • A chronic cholestatic liver disease of unknown etiology


  • Secondary sclerosing causes: choledocholithiasis, biliary tract surgery, AIDS cholangiopathy, chemotherapy, OLT (ischemia, CMV)


PATHOPHYSIOLOGY:



  • Idiopathic diffuse inflammation and fibrosis of the entire (intra- and extrahepatic) biliary tree


  • Frequent association with other diseases:



    • Ulcerative colitis >> Crohn’s disease; Rarely scleroderma, MCTD


  • DDX: Other causes of chronic cholestasis:



    • Biliary stones, Iatrogenic strictures, Tumors; Drug-induced: phenothiazines, estrogens, etc.


CLINICAL MANIFESTATIONS/PHYSICAL EXAM:



  • Pruritus and Fatigue


  • RUQ pain and Anorexia


  • Cholangitis (recurrent fever, RUQ pain, and jaundice) may occur


  • Cholangiocarcinoma superimposed


  • Liver is enlarged and firm to palpation


  • Late symptoms: those of end-stage liver disease: GIB, ascites, PSE


LABORATORY STUDIES:

Tags:
Aug 24, 2016 | Posted by in GASTROENTEROLOGY | Comments Off on Primary Sclerosing Cholangitis (PSC)

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