Recurrent Primary Biliary Cirrhosis
Rish K. Pai, MD, PhD
Key Facts
Clinical Issues
Prevalence increases progressively post transplantation
30-50% recurrence within 10 years
Most are found to have recurrent disease on protocol liver biopsies
Positive antimitochondrial antibody is less useful in diagnosis of recurrent PBC
Excellent prognosis; rarely progresses to cirrhosis
Microscopic Pathology
Histologic features are similar to native liver
Periductal, epithelioid, nonnecrotizing granulomatous reaction (definitive for recurrent disease if seen)
Cholate stasis may be seen as bile ducts are progressively lost
Features can be patchy within core biopsy with some portal tracts being completely normal
 The bile duct in this portal tract is infiltrated by lymphocytes (lymphocytic cholangitis  ), a finding seen in PBC. No granuloma is associated with this injured bile duct. |
 In this example of recurrent PBC, a well-formed granuloma is seen adjacent to a bile duct infiltrated by lymphocytes  . |
TERMINOLOGY
Abbreviations
Primary biliary cirrhosis (PBC)
Definitions
Recurrence of autoimmune disease affecting interlobular and septal bile ducts
CLINICAL ISSUES
Presentation
Recurrent PBC occurs in 10-50% of patients
Prevalence increases progressively post transplantation
Rare within 1 year post transplantation
10-30% within 5 years
30-50% within 10 years
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
