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Pathology

Destruction of intralobular bile ducts of the liver, this causes cholestasis resulting eventually in cirrhosis

Aetiology

Autoimmune condition, commonest in women aged 30 to 65
Associated with other autoimmune conditions

Signs

Scratch marks due to pruritus, jaundice, xanthoma due to hyperlipidaemia, if progresses to cirrhosis patients may have signs of chronic liver disease

Symptoms

Often diagnosed when patient is asymptomatic with abnormal LFTs, other symptoms include lethargy, pruritus (often early symptom). 

Investigations

Bloods: LFTs show raised bilirubin and ALP. 
Auto-Antibody Screen:  Antimitochondrial antibody (AMA) positive
Imaging: Ultrasound Abdomen may be normal, cirrhosis if advanced

Treatment

Medical: Ursodeoxycholic acid, steroids 
Surgical: Liver transplantation if cirrhotic.

Prognosis

If respond to Ursodeoxycholic acid  - normal life expectancy
Hepatocellular carcinoma 

Key Facts

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Key References

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