Internal Medicine

0521779407-17 CUNY1086/Karliner 0 521 77940 7 June 13, 2007 7:57

1210 Primary Biliary Cirrhosis

Imaging

■abdominal ultrasound or CT is not diagnostic

■early disease: usually normal; excludes biliary obstruction

■MRCP utilized when the diagnosis of PBC is uncertain to visualize

biliary tree (diagnostic ERCP if MRI contraindicated)

■advanced disease: hepatomegaly, nodular liver; venous collaterals

suggestive of portal hypertension

differential diagnosis

■autoimmune hepatitis

■autoimmune cholangitis

■other causes of obstructive jaundice (stones, benign/malignant

strictures)

■drug-induced cholestasis (estrogen, androgenic steroids)

■granulomatous hepatitis (including sarcoidosis)

■idiopathic adult ductopenia

management

What to Do First

■attempt to establish a firm diagnosis of PBC before treatment

General Measures

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

out biliary obstruction, exclude other hepatic diagnoses

■identify and manage the specific complications of end-stage liver

disease as they arise and complications of chronic cholestasis

■adjust or avoid potentially hepatotoxic medications

specific therapy

■indicated for any patient diagnosed with PBC and abnormal liver

enzymes:

➣ursodeoxycholic acid 13–15 mg/kg daily

■side effects and complications

➣ursodeoxycholic acid: diarrhea, rash, nausea, arthralgias

Specific Therapy for Complications Associated with PBC

■indications for treatment

➣cirrhosis/end stage liver disease: consider liver transplantation

for patients with CTP score=7; PBC may recur in transplanted

liver

➣pruritus: cholestyramine, 4 grams QID, spaced 4 hours apart from

other oral medications