Autoimmune hepatitis

Incidence

Age

Sex - F > M

Geography

Aetiology
- associated with other autoimmune diseases: pernicious anaemia, thyroiditis, Coomb's positive haemolytic anaemia
- associated with HLA-I B8, HLA-II DR3 and DR52a

- ? environmental agent causes an autoimmune response to liver antigens in a sensitive person?
- progressive necroinflammatory process -> fibrosis and cirrhosis
- defect of suppressor T-cells - primary or secondary?

Note:
- many drugs can cause a similar picture to autoimmune hepatitis
- e.g. methyldopa, isoniazid, ketoconazole, nitrofurantoin
- alcohol can produce a picture of chronic hepatitis
- improvement follows drug withdrawal

Presentation
History
- commonly asymptomatic, presenting with abnormalities of liver biochemistry
- 25% present as an acute hepatitis, with jaundice and very elevated aminotransferases

Examination
- may present with signs of chronic liver disease on examination
- hepatomegaly, cutaneous striae acne, hirsuites, bruises
- ± ascites
- may be features of autoimmune disease - fever, migratory polyarthritis, glomerulonephritis, pleurisy, pulmonary infiltration or fibrosing alveolitis

Investigations
LFTs
- high AST/ALT
- raised ALP and bilirubin
- high serum y-globulins

FBC
- mild normochromic normocytic anaemia
- thrombocytopenia
- leukopenia

Clotting
- PT often prolonged

Antibodies
Type I autoimmune hepatitis - ANA / anti-smooth muscle / anti-mitochondrial
Type II autoimmune hepatitis - anti-LKM1 / anti-liver cytosol

Liver biopsy
- changes of chronic hepatitis

Macro - 1/3 have cirrhosis on presentation

Micro - interface hepatitis - more severe in untreated patients
- lymphoid follicles

Staging

Serum markers

Management
Medical
- prednisolone 30mg daily for 2 weeks
- followed by maintenance dose 10-15mg daily + azathioprine 1-2 mg/kg daily

Surgical
- liver transplantation is indicated if medical treatment fails
- disease can recur

Prognosis - steroid+azathoprine induce remission in 80%
- those with cirrhosis are more likely to relapse when treatment ends - hence more likely to require indefinite therapy

Complications

Hepatobiliary medicine

Main Page

Incidence
Age
Sex	- F > M
Geography
Aetiology	- associated with other autoimmune diseases: pernicious anaemia, thyroiditis, Coomb's positive haemolytic anaemia - associated with HLA-I B8, HLA-II DR3 and DR52a - ? environmental agent causes an autoimmune response to liver antigens in a sensitive person? - progressive necroinflammatory process -> fibrosis and cirrhosis - defect of suppressor T-cells - primary or secondary? Note: - many drugs can cause a similar picture to autoimmune hepatitis - e.g. methyldopa, isoniazid, ketoconazole, nitrofurantoin - alcohol can produce a picture of chronic hepatitis - improvement follows drug withdrawal
Presentation	History - commonly asymptomatic, presenting with abnormalities of liver biochemistry - 25% present as an acute hepatitis, with jaundice and very elevated aminotransferases Examination - may present with signs of chronic liver disease on examination - hepatomegaly, cutaneous striae acne, hirsuites, bruises - ± ascites - may be features of autoimmune disease - fever, migratory polyarthritis, glomerulonephritis, pleurisy, pulmonary infiltration or fibrosing alveolitis
Investigations	LFTs - high AST/ALT - raised ALP and bilirubin - high serum y-globulins FBC - mild normochromic normocytic anaemia - thrombocytopenia - leukopenia Clotting - PT often prolonged Antibodies Type I autoimmune hepatitis - ANA / anti-smooth muscle / anti-mitochondrial Type II autoimmune hepatitis - anti-LKM1 / anti-liver cytosol Liver biopsy - changes of chronic hepatitis
Macro	- 1/3 have cirrhosis on presentation
Micro	- interface hepatitis - more severe in untreated patients - lymphoid follicles
Staging
Serum markers
Management	Medical - prednisolone 30mg daily for 2 weeks - followed by maintenance dose 10-15mg daily + azathioprine 1-2 mg/kg daily Surgical - liver transplantation is indicated if medical treatment fails - disease can recur
Prognosis	- steroid+azathoprine induce remission in 80% - those with cirrhosis are more likely to relapse when treatment ends - hence more likely to require indefinite therapy
Complications