AUTOIMMUNE LIVER DISEASES Flashcards
Which group are predominantly affected by primary biliary cirrhosis?
Women in their 4th to 6th decade
Female:male ratio of 10:1
What are the clinical features of primary biliary cirrhosis?
Pruritus Jaundice Hepatomegaly Clubbing Dark urine Pale stools Xanthoma Bone disease - reduced vitamin D absorption
What investigations would you order in someone presenting with suspected primary biliary cirrhosis?
LFTs
Autoantibody screen
Cholesterol level
Liver biospy
What would LFTs of someone with primary biliary cirrhosis show?
Raised ALP Derangement of other enzymes when cirrhosis occurs Synthetic function (INR and albumin) is usually preserved until late disease
What is the autoantibody that we look for to diagnose primary biliary cirrhosis?
Antimitochondrial antibodies
Apart from anti-mitochondrial antibodies, what are the antibodies often detected in primary biliary cirrhosis?
Anti-smooth muscle antibodies and ANA
Why does serum cholesterol increase in primary biliary cirrhosis?
Cholestasis leading to lack of cholesterol excretion. Cholesterol is normally excreted in bile.
What does the liver biopsy of someone with primary biliary cirrhosis show?
Characteristic picture of lymphocytic infiltration of around the portal tract, together with plasma cells and occasionally granulomas, resulting bile duct fibrosis, progressing to cirrhosis.
What are the extra-hepatic autoimmune diseases associated with primary biliary cirrhosis?
Rheumatoid arthritis
Sjogren’s syndrome
What are the complications of primary biliary cirrhosis?
Cirrhosis and hepatic failure
Associated with membranous glomerulonephropathy and renal tubular acidosis
Increased oesophageal varices as a result of portal hypertension
What is the prognosis for someone recently diagnosed with primary biliary cirrhosis?
Median survival is 12 years
Death is from progressive liver failure or complications
How are patients with primary biliary cirrhosis managed?
Mainly symptomatic treatment
Ursodeoxycholic acid has been shown to make moderate improvement
Ciclosporin, azathioprine and colchicine have also some effect in some patients but have big side effects.
Correct vitamin deficiencies (A, D and K)
Liver transplant is indicated in severe disease.
Treatment of hyperlipidaemia is not usually required.
What disease is most commonly associated with primary sclerosing cholangitis?
Ulcerative cholitis (60-80% of PSC cases)
Is primary sclerosing cholangitis more common in males or females?
Males - 3:1
What are the clinical features of primary sclerosing cholangitis?
Symptoms of IBD
Abdominal pain and jaundice with or without pruritus
Complications of cirrhosis
What do LFTs of someone with primary sclerosing cholangitis show?
Raised ALP
Raised GGT
Can be high bilirubin
Other than LFTs, what other investigations would you order in someone with suspected primary sclerosing cholangitis?
Autoantibody screen: pANCA
Liver biopsy
MRCP
ERCP
What autoantibodies are associated with primary sclerosing cholangitis?
pANCA
What would liver biopsy of someone with primary sclerosing cholangitis show?
A progressive fibrous obliterating cholangitis
Polymorph infiltration of bile ducts is characteristic.
What do MRCP or ERCP of a person with primary sclerosing cholangitis show?
Characteristic ‘bead’ like appearance can be seen due to multiple strictures throughout the bile ducts.
What are the complications of primary sclerosing cholangitis?
Those associated with cirrhosis and liver failure.
Also increases the risk of colorectal cancer in those with IBD
Up to 30% of patients may develop cholangiocarcinoma in the long term
How are patients with primary sclerosing cholangitis managed?
Ursodeoxycholic acid improves biochemical profile
Isolated strictures can be stented or balloon dilated.
Liver transplant is indicated in late stage disease.
Which group is most likely to develop autoimmune chronic active hepatitis?
Young women (less than 25)
How might autoimmune chronic active hepatitis present?
Either as an acute hepatitis with jaundice (25% of cases) or as chronic liver disease having been asymptomatic for years.
