Introduction An autoimmune disease in middle aged women resulting in an autoimmune destruction of bile ducts in the portal triads hampers the excretion of bile salts Incidence is 8 out of 100,000 people Presentation Symptoms chronic cholecystitis pruritis (itching) one of the first manifestations caused by excess bile salts deposited in the skin jaundice hyperlipidemia Evaluation Serology serum alkaline phosphatase levels greater than 1.5x the upper limit of normal typically normal serum total bilirubin levels Non-invasive imaging right upper quadrant ultrasound can be used to first distinguish between intra and extrahepatic biliary obstruction Anti-mitochondrial antibody 90% sensitive negative in primary sclerosing cholangitis Confirm with biopsy focal duct obliteration with granuloma formation Differential Polycythemia vera, atopic dermatitis, uremia secondary to hemodialysis, Treatment Liver transplant Supportive cholestyramine therapy is used to bind salts in the intestine enhancing their excretion decreases puritis Ursodeoxycholic acid first line treatment decreases symptoms increases transplant-free time