Autoimmune Hepatitis Flashcards
Definition
Rare, chronic autoimmune liver disease which can progress to cirrhosis
Antibodies associated with T1 AIH
Type 1 autoantibodies:
-Anti-nuclear antibodies (ANA)
-Anti-smooth muscle antibodies (ASMA)
-Anti soluble liver antigen (anti-SLA/LP)
Antibodies associated with T2 AIH
Type 2 autoantibodies:
- Anti-liver kidney microsomes-1 (anti-LICM1)
- Anti-liver cytosol antigens type 1 (anti-LC1)
Epidemiology
FEMALE
Age:
- Type 1 AIH = Adults
- Type 2 AIH = Children
Risk factors
HLA association:
- Type 1 AIH = HLA DR3, DR4
- Type 2 AIH = HLA DQB1, DRB1
Aetiology
Unclear and seems to be due to an interplay between environmental and genetic factors
Signs
Evidence of chronic liver disease:
- Jaundice
- Spider telangiectasia
- Gynaecomastia
- Splenomegaly
Evidence of acute liver failure:
- Jaundice
- Ascites
- Variceal bleed
- Encephalopathy
Symptoms
Fatigue
Arthralgia
Weight loss
Nausea
Amenorrhoea
Diagnosis
LFTs: elevated ALT and AST with normal or mildly elevated ALP. Bilirubin may also be raised
Immunoglobulins: IgG
Liver biopsy: inflammation of hepatocytes at the junction of the portal tract and hepatic parenchyma = piecemeal necrosis
Viral screen
Autoimmune screen: ANA, anti-SMA. Anti-SLA/LP, anti-LKM1, Anti-LC1
Management
FIRST LINE:
Immunosuppression: PREDNISOLONE and AZANTHIOPRINE
SECOND LINE: Transplantation
Complication
Cirrhosis
Osteoporosis
Cushing’s
Hepatocellular