9 terms
Primary Billiary Cholangitis
Which bile ducts does PBC affect?
Small intralobular bile ducts
Which antibody is most specific for PBC?
AMA (98% specific)
ANA also commonly positive
ANA also commonly positive
Which immunoglobulin tends to be raised in PBC?
IgM
Who gets PBC?
Middle aged women
95% women, most often 30-65 years old
95% women, most often 30-65 years old
Classic presentation of PBC?
Usually asymptomatic and diagnosed based on deranged LFTs
If symptomatic:
- Fatigue
- Pruritis
- Skin hyperpigmentation/dryness
- Xanthelasma/xanthomata
- Right upper quandrant discomfort
Can progress to chronic liver disease
- Jaundice, hepatomegaly, signs of portal hypertension (splenomegaly, ascites, varices)
If symptomatic:
- Fatigue
- Pruritis
- Skin hyperpigmentation/dryness
- Xanthelasma/xanthomata
- Right upper quandrant discomfort
Can progress to chronic liver disease
- Jaundice, hepatomegaly, signs of portal hypertension (splenomegaly, ascites, varices)
Which diseases are classically associated with PBC?
- Sjogren's (affects 65-80% of people with PBC)
- Thyroid disease (10-15%)
- Limited cutaneous scleroderma (5-15%)
- Classic rheumatoid arthritis (5-10%)
- Thyroid disease (10-15%)
- Limited cutaneous scleroderma (5-15%)
- Classic rheumatoid arthritis (5-10%)
What pattern do you expect to see in LFTs of a patient with PBC?
Predominantly cholestatic picture
- ALP>1.5 x upper limit of normal
Mild transaminitis (ie. ^AST and ALT)
- ALP>1.5 x upper limit of normal
Mild transaminitis (ie. ^AST and ALT)
Management of PBC?
Ursodeoxycholic acid
Supportive management for itch: antihistamines, cholestyramine
If decompensated cirrhosis, the only definitive management is liver transplant
Supportive management for itch: antihistamines, cholestyramine
If decompensated cirrhosis, the only definitive management is liver transplant
What screening should you do in a patient with PBC?
TSH - annual
Lipids
DEXA - every 6 months
Vitamin D (vitamin A and K as well if bili >20) - annual
Lipids
DEXA - every 6 months
Vitamin D (vitamin A and K as well if bili >20) - annual