Primary Biliary Cholangitis (PBC) Flashcards
What is primary biliary cholangitis?
Primary biliary cholangitis is an autoimmune disease of the liver.
It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up in the liver, a condition called cholestasis
Who is PBC most commonly seen in?
- Females
- 40-50
- Hx of AI disease
What causes the damage to interlobular bile ducts in primary biliary cholangitis?
A chronic inflammatory process causing progressive cholestasis.
What is the classic presentation of primary biliary cholangitis?
Itching in a middle-aged woman.
What autoimmune condition is seen in up to 80% of patients with primary biliary cholangitis?
Sjogren’s syndrome.
- Chronic autoimmune disorder that primarily affects the glands - leading to dry eyes and dry mouth, but can also affect other parts of the body
List three other associations with primary biliary cholangitis.
- Rheumatoid arthritis
- Systemic sclerosis
- Thyroid disease
What are the early clinical features of primary biliary cholangitis?
- May be asymptomatic
- Fatigue
- Pruritus
What is cholestatic jaundice?
A clinical feature of primary biliary cholangitis.
What skin changes may occur in primary biliary cholangitis?
- Hyperpigmentation
- Xanthelasmas
- Xanthomata
What percentage of patients with primary biliary cholangitis experience right upper quadrant pain?
Around 10%
What is the most specific antibody found in primary biliary cholangitis?
Anti-mitochondrial antibodies (AMA) M2 subtype.
In what percentage of patients are smooth muscle antibodies found in primary biliary cholangitis?
30%
What laboratory finding is commonly raised in primary biliary cholangitis?
Serum IgM.
What imaging techniques are required before diagnosing primary biliary cholangitis?
- MRCP
- Ultrasound
- MRI
What is the first-line treatment for primary biliary cholangitis?
Ursodeoxycholic acid.
What medication is used to treat pruritus in primary biliary cholangitis?
Cholestyramine.
What type of vitamin supplementation is important in the management of primary biliary cholangitis?
Fat-soluble vitamin supplementation.
What are some complications of primary biliary cholangitis?
- Cirrhosis
- Portal hypertension
- Osteomalacia
- Osteoporosis
- Increased risk of hepatocellular carcinoma
