76. Autoimmune Hepatitis, NAFLD and Wilson's Disease Flashcards
What is autoimmune hepatitis?
Inflammatory liver disease characterised by a normal T cells and autoantibodies directed against hepatocytes surface antigens
Who typically gets autoimmune hepatitis?
Young or middle aged woman
10-30 years old
40 years old
How does autoimmune hepatitis present?
Acute hepatitis
Signs of autoimmune conditions (fever, malaise, urticarial rash, polyarthritis, pleurisy, pulmonary infiltration, glomerulonephritis)
How is autoimmune hepatitis classified?
Type 1- woman under 40, antismooth msucle antibodies positive in 80%. Raised IgG. Good response to immunosuppression
Type 2- more often seen in children, commonly leads to cirrhosis and is less treatable. Typically anti-liver/kidney microsomal type 1 positive
How do you test for autoimmune hepatitis?
Raised serum bilirubin, AST,ALT,ALP
Positive antibodies
Liver biopsy- mononuclear infiltrate of portal and periportal areas
How do you treat autoimmune hepatitis?
Predinosolone- 30mg/d for 1 month. Reduce 5mg a mont until maintenance dose is achieved.
Corticosteroids can be stopped after 1 year but relapse is likely
What are the associations of autoimmune hepatitis?
Pernicious aneamia Ulcerative colitis Glomerulonephritis Auto-immune thyroiditis Autoimmune heamolysis Diabetes mellitus PSC
How do you initially investigate fatty liver disease?
Look for other causes
USS
check for associated metabolic disorders (high lipids)
What are the risk factors for progression to non-alcoholic fatty liver disease?
Older age
Obesity
Diabetes mellitus
Non alcoholic steatohepatitis
How do you treat NAFLD?
Lose weight
Address cardiovascular risk
Avoid alcohol consumption
Vitamin E may improve fibrosis risk
What is Wilson’s disease?
Inherited disorder, caused due to failure to excrete copper and deposits in the liver.
APTase/ATP7B is the transporter affected
How does Wilson’s disease present?
Children present with hepatitis, cirrhosis, fulminant liver failure
Young adults- CNS symptoms, parkinsonisms, low mood, low IQ, delusions, mutism
What are the signs of Wilson’s disease?
Copper in Iris
Heamodialysis, blue nails,arthritis
How do you test for Wilson’s disease?
Urine 24 hour. Operating LFT’S Serum copper Serum caeroploasmin Genetics test Liver biopsy
How do we manage Wilson’s disease?
Avoid food with high copper content
Lifelong penicillinamine
Liver transplant
Screen siblings
