Primary Biliary Cirrhosis
Matthew M. Yeh, MD, PhD
Key Facts
Terminology
-
Idiopathic chronic cholestatic autoimmune liver disease in which intrahepatic bile ducts are progressively destroyed by nonsuppurative inflammation
Clinical Issues
-
Middle-aged to elderly women
-
Insidious onset with pruritus
-
Fatigue, jaundice, associated autoimmune disorders
-
Positive antimitochondrial antibodies (AMA)
-
Minority of cases are AMA(-)
-
-
Elevation of GGT, alkaline phosphatase, bilirubin
-
Ursodeoxycholic acid (UDCA) is treatment of choice
Microscopic Pathology
-
Florid duct lesion
-
Bile duct injury
-
Bile ductular reaction
-
Chronic cholestasis
-
Portal-based fibrosis
-
Copper stain highlights accumulated copper in hepatocytes (copper is normally excreted in bile)
Top Differential Diagnoses
-
Primary sclerosing cholangitis (PSC)
-
Secondary biliary obstruction
-
Drug-induced chronic cholestasis
-
Sarcoidosis
-
Autoimmune hepatitis
-
Some patients have autoimmune hepatitis-PBC overlap syndrome with features of both
-
TERMINOLOGY
Abbreviations
-
Primary biliary cirrhosis (PBC)
Definitions
-
Chronic cholestatic autoimmune disease in which intrahepatic bile ducts are progressively destroyed by nonsuppurative inflammation
ETIOLOGY/PATHOGENESIS
Unknown
-
Most likely multifactorial
CLINICAL ISSUES
Epidemiology
-
Incidence
-
Up to 30/100,000 in Scandinavia and parts of North America
-
Much more prevalent among individuals of North European descent
-
-
Distinctly rare in Asians
-
-
Age
-
Middle-aged to elderly (40-60 years old)
-
-
Gender
-
Predominantly women (F:M = 9:1)
-
Presentation
-
Insidious onset with pruritus (most common), fatigue, jaundice, associated autoimmune disorders
Laboratory Tests
-
Positive antimitochondrial antibodies (AMA)
-
Elevation of GGT &/or alkaline phosphatase
-
Elevated bilirubin
-
Elevated IgM
-
Mildly elevated transaminases
Treatment
-
Surgical approaches
-
Liver transplantation
-
-
Drugs
-
Ursodeoxycholic acid (UDCA)
-
Prognosis
-
Chronic, progressive disease
MICROSCOPIC PATHOLOGY
Histologic Features

Stay updated, free articles. Join our Telegram channel

Full access? Get Clinical Tree

