Sclerosis of bile duct is seen in -

Ans. is 'a' i.e., Primary sclerosing cholangitis Features of the Bile Duct Disorders Primary Biliary CirrhosisSecondary Biliary CirhossisPrimary Sclerosing CholangitisCausePossibly autoimmuneBiliary atresia, gallstones, stricture, cancer of pancreatic headAutoimmune, usually associated with inflammatory' bowel diseaseSex predilectionFemale to male ; 6:1NoneFemale to male; 1:2Distributionintrahepatic bile duct obstructionExtrahepatic bile duct obstructionExtra + ntra hepatic duct affectedLab, findingsSame as secondary' biliary cirrhosis with elevated serum IgM anlimstoehondrial antibody| conjugated bilirubin, | serum alkaline phosphatase with increased cholesterolSame as secondary' biliary cirrhosis with hypergammaglobulinemia (|IgM), atypical p-ANCA (+)Histological findingsDense lymphocytic infiltrate in porta! tracts with granulomatous destruction of bile ductsBile stasis in bile ducts, bile ductules proliferation with surrounding neutrophils, portal tract edemaPeriductal portal tract fibrosis (onion skin fibrosis), segmental stenosis of extra and intrahepatic bile ductsAn important feature is Atypical p-ANCA (+) is seen with primary1 sclerosing cholangitis but that this antibody is directed against a nuclear envelope protein and not myeloperoxidase seen with typical p-ANCA antibodies.