Primary Biliary Cirrhosis/Primary Biliary Cholangitis (PBC)

WHAT IS IT?

Primary biliary cirrhosis/primary biliary cholangitis (PBC) is an autoimmune disease that causes progressive destruction of the bile ducts.

WHAT CAUSES IT?

Autoimmune antibodies seem to be responsible for mediating this condition

WHY DO WE CARE MEDICALLY ABOUT THIS CONDITION?
 
WHAT IS THE INTIAL PRESENTATION FOR THIS CONDITION?

Patient Issues/Chief Complaints:

  • Abdominal pain (often RUQ)
  • Jaundice 
  • Pruritus 
  • Fatigue 

Detection On Medical Workup:

 

WHAT ARE IMPORTANT ELEMENTS OF THE MEDICAL HISTORY?

Risk Factors:

Female sex, autoimmune disease (scleroderma, rheumatoid arthritis, Sjogrens)

History Of Present Illness (HPI):

 

WHAT ARE IMPORTANT FEATURES OF THE PHYSICAL EXAM?

Vital Signs:

 

CLINICAL WORKUP: SERUM STUDIES

Auto-Antibody Testing:

  • Anti-mitochondrial antibodies will be present most of the time int he patient serum (both sensitive and specific)
CLINICAL WORKUP: IMAGING

 

CLINICAL WORKUP: HISTOLOGY

Liver Biopsy:

  • Granulomatous bile duct destruction
WHAT IS OUR THRESHOLD FOR DIAGNOSING THIS CONDITION?

 

WHAT ELSE SHOULD WE CONSIDER WHEN THIS DIAGNOSIS IS MADE?

 

HOW DO WE MANAGE A PATIENT WITH THIS DIAGNOSIS?

 

WHAT IS THE PROGNOSIS FOR A PATIENT WITH THIS DIAGNOSIS?

 

COULD THIS CONDITION HAVE BEEN PREVENTED?

 

ARCHIVE OF STANDARDIZED EXAM QUESTIONS 

This archive compiles standardized exam questions that relate to this topic.

FURTHER READING

DynaMed

UpToDate

 

 

Page Updated: 01.29.2017