Internal Medicine

0521779407-17 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:19

Primary Sclerosing Cholangitis 1215

➣early disease: often asymptomatic and associated with few phys-

ical signs; often detected after routine chemistry panel shows

increased LFTs

➣advanced disease: liver failure

tests

Laboratory

Basic Studies: Blood

■early disease: cholestatic pattern

➣increased alkaline phosphatase and GGT, mild increased

AST/ALT, normal or transiently increased bilirubin

➣serology: autoantibodies nonspecific, nondiagnostic; ANA, SMA

positive in 50%; p-ANCA positive in majority

■advanced disease: increased bilirubin and INR; hypoalbuminemia,

malnutrition

Imaging

■ultrasound or CT scan: nonspecific/nondiagnostic

➣early disease: normal or hepatomegaly; dilated ducts may be sug-

gestive of malignancy

➣advanced disease: hepatomegaly, venous collaterals, signs of

portal hypertension

■ERCP

➣traditional “gold standard” for diagnosis of PSC: stricturing and

dilation of the biliary ducts; ductal pseudodiverticula considered

pathognomic

■MRCP

➣replacing ERCP for diagnosis of PSC (less invasive, fewer compli-

cations, excellent images; intrahepatic ducts less well visualized)

Liver Biopsy

■helpful, but not necessary for diagnosis of PSC; excludes other or

concurrent diagnoses; stages the disease

➣early disease: bile duct epithelium infiltrated with inflammatory

cells arranged in a concentric manner, previously named peri-

cholangitis

➣advanced disease: portal-to-portal bridging fibrosis; biliary duc-

tular proliferation, periductular sclerosis and stenosis

differential diagnosis

■secondary causes of sclerosing cholangitis: choledocholithiasis,

chronic infections due to parasites or opportunistic organisms,