Pathology: Chronic hepatitis of autoimmune origin
Aetiology: Unknown, association with other autoimmune diseases
Symptoms: Lethargy, pruritus
Signs: Asymptomatic, jaundice
Investigations: Bloods: LFTs show raised bilirubin and ALT
Auto-Antibody Screen: Anti-smooth muscle and Anti-nuclear (ANA) positive
Imaging: Ultrasound Abdomen may be normal, cirrhosis if advanced
Treatment: Medical: Steroids and immunosuppression with azathioprine
Complications: Cirrhosis, liver failure
Prognosis: 50% of patients will die within 10 years of disease onset without treatment.
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
Symptoms: Often diagnosed when patient is asymptomatic with abnormal LFTs, other symptoms include lethargy, pruritus (often early symptom).
Signs: Scratch marks due to pruritus, jaundice, xanthoma due to hyperlipidaemia, if progresses to cirrhosis patients may have signs of chronic liver disease
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
Complications: Hepatocellular carcinoma
Prognosis: If respond to Ursodeoxycholic acid - normal life expectancy
Pathology: Autoimmune disorder characterised by inflammation and stricture formation of the intra and extra hepatic bile ducts
Aetiology: Autoimmune, higher incidence in men, associated with IBD. Ulcerative colitis has a higher prevalence of disease than Crohn’s.
Symptoms: Pruritus, lethargy, symptoms of IBD
Signs: Excoriations from pruritus, jaundice if late
Investigations: Bloods: LFTs show raised bilirubin and ALP
Auto-Antibody Screen: ANCA positive
Imaging: Ultrasound Abdomen is usually normal, can show bile duct dilatation
Treatment: Medical: ERCP to dilate ducts
Surgical: Liver transplantation
Complications: Increased risk of cholangiocarcinoma, colonic carcinoma and cholangitis
Prognosis: Median survival 12 years from diagnosis
