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Autoimmune hepatitis (AIH) (Associations (PSC/PBC, Pernicious anaemia, UC,…
Autoimmune hepatitis (AIH)
Definition
Autoimmune inflammatory
liver disease of unknown cause
Epidemiology
Onset in children or young/middle
aged women (age 10-30y or >40y)
Pathophysiology
Mechanism
Unknown trigger
Abs against hepatocyte antigens
Can present as acute, fulminant or chronic hepatitis
Classification
Type I
Most common type; young females
Anti-smooth muscle Abs +ve 80%, ANA +ve 10%
Responsive to immunosuppression
Type II
Children
Anti-liver/kidney miscrosomal Abs +ve
Less treatable, progression to cirrhosis
Type III
Soluble liver antigen/liver-pancreas antigen Abs +ve
Clinically like type I
Clinical
presentation
Abdominal pain (RUQ)
Signs of
autoimmune
disease
Malaise
Urticaria
Fever
Polyarthritis
Pleurisy
Asymptomatic
Jaundice
Diagnosis
Investigations
Bloods
FBC (anemia, thrombocytopenia, leukopenia)
CRP/ESP (elevated), U+Es (may be deranged)
LFTs (elevated bilirubin, AST, ALT, ALP, total protein)
Immune screen (+ve autoantibodies, high IgG)
Liver biopsy
Mononuclear infiltrate,
necrosis, fibrosis, cirrhosis
Imaging
Abdo USS: inflamed liver
MCRP (exclude PSC)
Urine
Dipstick (blood/leukocytes if GN)
PCR/ACR
Bedside
Obs (any fever)
Examination
Abdo exam
Jaundice, hepatomegaly, splenomegaly,
tender RUQ if acute, signs of decompensated
chronic liver disease
History
DH
Recent meds, allergies
FH
AIH, PSC/PBC, metabolic disorder
PMH
Known medical conditions,
growth and development, vaccinations
SH
Living arrangements, occupation/school/nursery,
smoking, alcohol
PC/HPC
Jaundice, abdominal pain, fever,
malaise/fatigue, urticaria, joint pain
Management
Definitive
Conservative
Information, advice, support
Medical
Steroids
Indication: acute flare
E.g. prednisolone
immunosuppression
Indication: long-term
E.g. azathioprine
Surgical
Liver transplant
Indication: decompensated cirrhosis;
failure to respond to meds
Initial ABCDE
Complications
Cirrhosis
HCC
Acute/chronic liver failure
Associations
PSC/PBC
Pernicious anaemia
UC
GN
DM
Autoimmune thyroiditis