Primary Biliary Cirrhosis

Question 1

What is primary biliary cirrhosis?

Answer 1

Autoimmune origin disease characterised by destruction of intralobular bile ducts

Question 2

What are the clinical features of primary biliary cirrhosis?

Answer 2

Patients are initially often asymptomatic. Signs and symptoms are mainly due to the resulting cholestasis, liver cirrhosis, and portal hypertension.
Fatigue (usually the first symptom)
Marked generalized pruritus
Hyperpigmentation [6]
Hepatomegaly, dull lower margin, RUQ discomfort
Splenomegaly
Jaundice
Pale stool, dark urine
Maldigestion (may involve manifestations of deficiency of fat-soluble vitamins; e.g., osteoporosis) [2]
Xanthomas and xanthelasma [2]
Frequently associated with other autoimmune conditions [6]
Rheumatoid arthritis
Autoimmune thyroid disease, especially Hashimoto thyroiditis
Celiac disease
CREST syndrome
Sicca syndrome

Question 3

What are the diagnostic tests for primary biliary cirrhosis?

Answer 3

Laboratory tests
↑ Cholestasis parameters (ALP, γ-GT, conjugated bilirubin)
Transaminases (AST/ALT) are within normal limits or slightly elevated
Hypercholesterolemia
↑ Antimitochondrial antibodies (AMA) (> 95%)
↑ ANA (up to 70%)
↑ IgM
Imaging:
Abdominal ultrasound: to screen for mechanical bile obstruction
Magnetic resonance cholangiopancreatography: to confirm intrahepatic bile duct occlusion
Liver biopsy
Performed in case of suspicion for an overlap syndrome or uncertain diagnosis (e.g., if AMA are negative but clinical suspicion is strong)
Necessary for staging

Question 4

What is the d/d for PBC?

Answer 4

Autoimmune hepatitis
Primary sclerosing cholangitis (See “Differential diagnoses of cholestatic biliary disease” for details.)
Biliary obstruction (malignant or benign)
Drug-induced liver damage
Sarcoidosis

Question 5

What is the treatment for PBC?

Answer 5

No cure for PBC. Treatment consists of slowing disease progression and alleviating symptoms.

Liver transplant is the only cure.

First line therapy - ursodiol
Second line therapy - obeticholic acid

Question 6

What are the complications of PBC?

Answer 6

Malabsorption
Liver cirrhosis and portal hypertension
Osteoporosis