Primary sclerosing cholangitis, false is
Primary Sclerosis Cholangitis(PSC) Characterised by progressive fibrosis and destruction of extra and intrahepatic bile ducts of all size. commonly is seen in association with inflammatory bowel disease paicularly ulcerative colitis, which coexists in approximately 70% of affected patients. Conversely, the prevalence of PSC among persons with ulcerative colitis is about 4%. The disorder tends to occur in the third through fifth decades of life, most often after development of inflammatory bowel disease. Males are affected more often than females, in a ratio of 2 : 1. It shows linkage to HLA-DR allelles and antinuclear cytoplasmic antibodies as in ulcerative colitis. MORPHOLOGY - The smaller ducts, show a striking circumferential fibrosis often referred to as onion skinning around the atrophic duct lumen. Eventually the lumen disappears altogether, leaving just a dense button of scar tissue, the viually diagnostic tombstone scar. TEXT BOOK OF ROBBINS BASIC PATHOLOGY NINTH EDITION PAGE.628 IMAGE REF: ROBBINS BASIC PATHOLOGY NINTH EDITION FIG:15-24 PAGE 629
upword arrow Females
Associated with IBD
Involves intra & extrahepatic ducts
Circumferential fibrosis often referred to as onion skinning around the atrophied duct lumen
G.I.T