Internal Medicine

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

1216 Primary Sclerosing Cholangitis

ischemic bile duct injury, drug-induced sclerosing cholangitis (flox-

uridine)

■sclerosing variant of cholangiocarcinoma

■idiopathic adulthood ductopenia

■autoimmune cholangitis

■drug-induced cholestasis

management

What to Do First

■establish diagnosis by MRCP or ERCP; exclude secondary causes

of sclerosing cholangitis, cholangiocarcinoma; look for evidence of

inflammatory bowel disease (if patient has no colonic symptoms or

has not been previously evaluated do flexible sigmoidoscopy, biop-

sies)

General Measures

■use liver biopsy, imaging studies to assess disease severity and rule

out biliary obstruction or cirrhosis, exclude other diagnoses

■therapeutic ERCP if evidence of biliary ductular dilatation on ultra-

sound, CT or sudden change in liver function tests; do biliary brush-

ings/biopsies to rule out cholangiocarcinoma

■identify and manage the silent complications of chronic cholestasis

■adjust or avoid potentially hepatotoxic medications

■optimize therapy of underlying inflammatory bowel disease

specific therapy

■no proven medical therapy; UDCA commonly used in high doses

(25–30 mg/kg/day)

■therapy directed at treating complications of the disease

follow-up

Routine

■early disease: patients should be seen semi-annually and screened

for possible silent complications of PSC (i.e. assess bone mineral

density); manage biliary complications with therapeutic ERCP

■advanced disease: patients should be seen every 3–6 months and

■refer for liver transplantation (OLT) when minimal listing criteria are

met (CPT score≥7)

complications and prognosis

Complications

■cirrhosis/end-stage liver disease: refer for OLT, sooner rather than

later, to avoid complication of cholangiocarcinoma