Autoimmune hepatitis Flashcards
what is the definition of autoimmune hepatitis?
Autoimmune hepatitis (AIH) is a chronic inflammatory disease of the liver of unknown aetiology. It is characterised by the presence of circulating auto-antibodies with a high serum globulin concentration, inflammatory changes on liver histology, and a favourable response to immunosuppressive treatment
what is the epidemiology of autoimmune hep?
Prevalence is greatest among people with northern European ancestry who have a high frequency of human leukocyte antigen (HLA)-DR3 and HLA-DR4 markers
Female and children more commonly affected
what is the aetiology of autoimmune hep?
Unknown
what are the risk factors for autoimmune hep?
Female
Genetic predisposition
Immune dysregulation
what is the pathophysiology of autoimmune hep?
It is believed that in a genetically predisposed person, an environmental agent can trigger a pathogenic process leading to liver necrosis and fibrosis.
Auto-antigens have been implicated in the initiation of the cascade of events in AIH. If environmental agents are involved in triggering the disease, then molecular mimicry may come into play.
what are the key presentations of autoimmune hep?
Presence of risk factors Fatigue Anorexia Abdominal discomfort Hepatomegaly Jaundice encephalopathy
what are the signs of autoimmune hep?
Risk factors Anorexia Hepatomegaly Jaundice Encephalopathy Spider angiomata
what are the symptoms of autoimmune hep?
Fatigue Abdo discomfort Pruritus Arthralgia Nausea
what are the first line and gold standard investigations for autoimmune hep?
Aspartate transaminase - elevated Alanine transaminase - elevated Bilirubin - increased Gamma glutamyl transferase - increased Alkaline phosphatase - increased Serum globulin - increased Serum albumin - decreased Prothrombin time - prolonged
what are the differential diagnoses for autoimmune hep?
PBC, primary sclerosing cholangitis, chronic hep B, chronic hep C
how is autoimmune hep managed?
High dose corticosteroid, liver transplant
how is autoimmune hep monitored?
Progress is usually monitored by levels of serum aminotransferases, bilirubin, prothrombin time, albumin, and globulins (total or gamma globulin) measured on a monthly basis. Monitoring has to be continued upon achievement of remission, in order to detect possible relapse.
what are the complications of autoimmune hep?
Corticosteroid side effects: Osteoporosis Diabetes Hypertension Truncal obesity Growth impairment in children
what is the prognosis of autoimmune hep?
The natural history of AIH reveals that untreated AIH has a poor prognosis with a 5-year survival rate of 50% and 10-year survival rate of 10%
