Primary Biliary Cholangitis

University:
Western Sydney University
Course:
MEDI 2003 | Clinical Sciences 2
Academic year:

2024
Views:

332

Pages:

4
Author:

Mariam A. A.

Primary Biliary Cholangitis Definition Chronic autoimmune liver disease Immune cells slowly destroy bine ducts -> bile build up -> inflammation -> scarring -> cirrhosis -> liver failure Epidemiology Uncommon cause of chronic liver disease e More common in females Most diagnosed between 30 and 65 years Key Info Disease course is influenced by a dominant hilar or distal biliary stricture Clinical features Typical presentation Middle aged women presenting with fatigue, pruritus and laboratory evidence Icterus Prutitis Fatigue Most common Jaundice Skin hyperpigmentation Especially arms and trunk Splenomegaly Xanthelasma Sicca syndrome Kayser Fleischer rings Very rare RUQ discomfort Investigations LFTs ALT and AST elevation in most patients Elevated lipid and cholesterol levels Increase in ESR Must meet at least 2 of 3 of: Antimitochondrial antibodies ALP 1.5x the upper limit Nonsuppurative cholangitis and interlobular bile duct injury on histology Ultrasound Periportal hypoechoic band Thickened portal vein wall Heterogenous liver texture Left lobe enlargement Splenomegaly CT Biliary tree irregularities, ductal dilation or strictures MRI Biopsy – Ludwing and Scheuer system Stage Description Portal inflammation Stage 1 -> portal stage of Ludwig Bile duct abnormalities Periportal fibrosis Stage 2 -> periportal stage +/- periportal inflammation Prominent enlargement of portal tracts Septal fibrosis with active inflammatory infiltrate Stage 3 -> septal stage Passive pluricellular septa Stage 4 -> cirrhosis Nodules with various degrees of inflammation Management Aims: Slow disease progression rate Alleviate symptoms Ursodeoxycholic acid First line At 13 to 15 mg/kg orally daily If no adequate biochemical response after 12 months -> obeticholic acid or fibrates (eg fenofibrate), under the management of a liver specialist. Cholestyramine Can be used for pruritus management Complications Dyslipidaemia Fat soluble vitamin deficiency Metabolic bone disease Weight loss Fat soluble vitamin deficiency Night blindness Weak bones Poor skin development Anaemia Hypo coagulation -> bleeding Cirrhosis Associations Cholelithiasis Interstitial lung disease Autoimmune diseases e.g. Sjogren disease, autoimmune thyroiditis or scleroderma Pulmonary hypertension