Cause of pruritus in primary biliary cirrhosis?
Primary biliary cirrhosis- Characterized by chronic inflammation and fibrous obliteration of intrahepatic bile ductules. Associated with autoimmune disorders-CREST syndrome, Sicca syndrome, Autoimmune thyroiditis, Type-1 Diabetes mellitus and IgA deficiency. Mostly seen in females Age is 35-60 yrs C/F-Pruritis, jaundice, steatorrhoea, xanthelasma, hepatomegaly, splenomegaly, ecchymosis, osteomalacia. Cause of pruritis is due to vanilloid receptor Associated with cholesterol stones (not pigment stones) due to reduced bile acid synthesis . Lab findings: | Serum 5' nucleotidase activity | Gamma-glutamyl transpeptidase activity | Serum bilirubin levels | Serum lipid levels |ALP Confirmation test- Liver biopsy (Florid duct lesion (granuloma)) Treatment of choice is liver transplantation. Primary biliary cirrhosisnow called as Primary Biliary Cholangitis
TGR 4
Glucagon like peptide
Vanilloid receptor
Gastrin releasing peptide
JIPMER 2018