Primary Biliary Cirrhosis Flashcards
What is primary biliary cirrhosis. (2)
Chronic inflammation and destruction of the small and medium bile ducts.
It is a chronic, progressive cholestatic liver disease.
Who gets affected by primary biliary cirrhosis. (2)
80-90% women.
Age 30-60.
What are the clinical features of primary biliary cirrhosis. (9)
Fatigue. Pruritus. Arthralgia. Xanthelasma. Hepatomegaly. Splenomegaly. Jaundice (rare). Signs of chronic liver disease. Osteoporosis.
What is the antibody strongly associated with PBC.
AMA (antimitochondrial antibodies).
What is the characteristic histological finding in PBC.
Granulomatous inflammation of the portal tracts, leading to progressive damage and eventually loss of the small and middle sized bile ducts.
What happens in PBC after the damage of the bile ducts. (3)
Fibrosis, which in turn leads to cirrhosis of the liver, and portal hypertension.
What does PBC initially present with. (2)
Itching.
And possible tiredness.
What sort of onset does PBC have.
Insidious.
What parts of the world is PBC most common. (2)
Europe.
North America.
Where in the world is PBC rare. (2)
Africa.
Asia.
PBC is more common amongst _________
Smokers.
What other conditions is PBC associated with. (4)
Thyroid disease (hypothyroidism).
Sicca syndrome.
Systemic sclerosis.
Coeliac disease.
What gene is associated with PBC.
HLA-DR8
What does AMA target.
Pyruvate dehydrogenase complex.
What is the osteomalacia associated with PBC caused by.
Malabsorption of the fat soluble vitamins.
What is the osteoporosis associated with PBC caused by.
Hepatic osteodystrophy.
What is the prevalence of PBC.
4/ 100,000
What may be present on routine blood tests in a patient with undiagnosed PBC. (2)
AMA.
Raised serum alkaline phosphatase.
What is seen in 70% of patients with PBC.
Keratoconjunctivitis sicca.
What is keratoconjunctivitis sicca.
Dry eyes and mouth.
What type of condition is PBC.
Autoimmune.
What risk factors increase the likelihood of developing PBC. (6)
Family history (seen in 1-6%). Chronic UTIs. Smoking. Past pregnancy. Other autoimmune diseases. Use of nail polish/hair dye.
What are the fat soluble vitamins. (4)
A.
D.
E.
K.
Why is there malabsorption of fat soluble vitamins in PBC.
Due to cholestasis.
What are the complications of PBC. (5)
Those associated with cirrhosis.
Osteoporosis.
Malabsorption of fat soluble vitamins resulting from cholestasis causes osteomalacia.
Decreased bilirubin in the gut lumen (along with the cholestasis) results in coagulopathy.
Hepatocellular carcinoma.
