Autoimmune Disease of the Biliary Tract Flashcards
(18 cards)
What is autoimmune hepatitis?
Unknown aetiology
Seen in young females
Associated with
- HLA B8
- HLA- DR3
- Coeliac Disease
- Other autoimmune disease
What are clinical features of autoimmune hepatitis?
Can present as features of CLD
Acute hepatitis
- Deranged liver functions- ALT/AST 5-10times upper limit normal
- Jaundice
- fever
Can present with non specific symptoms e.g. fatigue, amenorrhoea
What is found on investigation of autoimmune hepatitis?
Deranged LFTs
INR elevated
Antibodies
Hypergammaglobulinaemia
What is Type 1 AH?
Affects adults and children
Antibodies- anti smooth muscle antibody +/- ANA
What is Type 2 AH?
Only in children
Anti Liver Kidney Microsomal antibody
What is type 3 AH?
Affects middle aged adults
Anti-soluble liver antigen antibodies
Normally good response to prednisolone
What is the management of AH?
Prednisolone/steroids
Azathioprine/immunosuppressants
Liver transplant
What is Primary Biliary Cirrhosis?
Chronic inflammation of the interlobular ducts resulting in cholestasis and eventually cirrhosis
What is Primary Biliary Cirrhosis associated with?
Sjogrens Syndrome
RA
Systemic sclerosis
Thyroid diseaseW
What are the clinical features of Primary Biliary Cirrhosis?
Middle aged woman with pruritis and fatigue
Raised ALP
Jaundice
Clubbing
Hepatosplenomegaly
Xanthelasma
What investigations are required in Primary Biliary Cirrhosis?
Anti-Mitochondrial antibodies M2
Raised IgM
Imaging
- required prior to diagnosis
- MRCP or RUQ US
What is the management of Primary Biliary Cirrhosis?
1) Ursodeoxycholic acid- reduces symptoms and slows disease progression
Cholestyramine can aid symptoms
Liver transplant
- esp once bili >100
What are complications of Primary Biliary Cirrhosis?
Liver cirrhosis- portal HTN, ascites and haemorrhage
Osteomalacia
Osteoporosis
Significant increased risk of HCC
What is Primary Sclerosing Cholangitis?
Inflammation of the intra and extra hepatic bile ducts with subsequent fibrosis
What conditions are associated with Primary Sclerosing Cholangitis?
Ulcerative Colitis
HIV
What are clinical features of Primary Sclerosing Cholangitis?
RUQ pain
Obstructive jaundice
Pruritis
Raised bili and ALP
Fatigue
Hepatosplenomegaly
What investigations are required for Primary Sclerosing Cholangitis?
PANCA can be positive
ERCP or MRCP are standard investigations
- show multiple biliary strictures- beaded appearance
Limited role of liver biopsy
What are the complications of Primary Sclerosing Cholangitis?
Cholangiocarcinoma
