{"type":"rich","version":"1.0","provider_name":"Transistor","provider_url":"https://transistor.fm","author_name":"Board Pearls","title":"Chapter 20, Ep 3 of 3: Primary Sclerosing Cholangitis and IgG4 Disease","html":"","width":"100%","height":180,"duration":925,"description":"Episode three works primary sclerosing cholangitis, the multifocal stricturing disease of the man with ulcerative colitis, as a recognition-and-surveillance problem, then covers its steroid-responsive mimic. MRCP not serology makes the diagnosis, the alkaline phosphatase is normal in nearly half of patients at any moment, and because there is no proven medical therapy the work is surveillance for three cancers. The chapter closes on IgG4-related sclerosing cholangitis, the older man with painless jaundice and a sausage-shaped pancreas who responds dramatically to corticosteroids and must not go to a Whipple, plus the two overlap syndromes. Cancer thresholds, the dominant stricture workup, and the high-dose ursodeoxycholic acid harm signal run throughout.\n \nTopics covered\n\nSclerosing cholangitis presentation and IBD association\nMRCP as the diagnostic test and role of ERCP\nNatural history and premalignant surveillance rationale\nCholangiocarcinoma surveillance and CA 19-9\nDominant stricture workup and brush cytology ceiling\nGallbladder and colorectal cancer thresholds\nNo proven therapy and high-dose UDCA harm\nIgG4-related sclerosing cholangitis and HISORt\nAutoimmune hepatitis overlap syndromes\n \n \nKey decisions\n\nDiagnose primary sclerosing cholangitis with MRCP, not serology, because a normal alkaline phosphatase occurs nearly half the time and the beaded pattern of multifocal short-segment strictures with intervening normal or dilated segments closes it; reserve ERCP for dominant stricture, cholangitis, or suspected cancer.\nSurveil for cholangiocarcinoma with annual MRCP plus CA 19-9 starting at diagnosis, interpreting the CA 19-9 by trajectory from the patient's baseline rather than the absolute number, since it is uninformative in Lewis-negative patients and rises with cholangitis.\nTreat every dominant stricture as cancer until proven otherwise with ERCP brushings, molecular testing for chromosomal polysomy, and cholangioscopy-directed biopsy, because brush...","thumbnail_url":"https://img.transistorcdn.com/-FuAdDBcPDLhEoUmroZKtOBRvuBn_FHPpYlh41hOnU4/rs:fill:0:0:1/w:400/h:400/q:60/mb:500000/aHR0cHM6Ly9pbWct/dXBsb2FkLXByb2R1/Y3Rpb24udHJhbnNp/c3Rvci5mbS9iNzlh/ZTU4Y2MzNWExMjQ5/MjA5OWMwMmI3ZTk5/NGFiZS5wbmc.webp","thumbnail_width":300,"thumbnail_height":300}