<prompt> All of the following are true about Primary Biliary Cirrhosis except?Increase 5- nucleotidaseMedian age of presentation is 50 yearsMost common cause of cholangitis in childrenPBC frequently associated with CREST syndrome  <response>: 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 CF-Pruritis, jaundice, steatorrhoea, xanthelasma, hepatomegaly, splenomegaly, ecchymosis, osteomalacia. 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 Treatment of choice is liver transplantation. 