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. Topics covered Sclerosing cholangitis presentation and IBD association MRCP as the diagnostic test and role of ERCP Natural history and premalignant surveillance rationale Cholangiocarcinoma surveillance and CA 19-9 Dominant stricture workup and brush cytology ceiling Gallbladder and colorectal cancer thresholds No proven therapy and high-dose UDCA harm IgG4-related sclerosing cholangitis and HISORt Autoimmune hepatitis overlap syndromes Key decisions Diagnose 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. Surveil 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. Treat every dominant stricture as cancer until proven otherwise with ERCP brushings, molecular testing for chromosomal polysomy, and cholangioscopy-directed biopsy, because brush cytology has a false-negative rate over fifty percent. Remove the gallbladder for polyps greater than eight millimeters, a lower threshold than the ten-millimeter general-population cutoff, and follow smaller polyps with ultrasound every six months. Perform colonoscopy at the time of sclerosing cholangitis diagnosis regardless of IBD duration, then every one to two years, because the combination enriches colorectal cancer risk several-fold and the diagnosis resets the surveillance clock. Do not use high-dose ursodeoxycholic acid above twenty-eight milligrams per kilogram per day, because a large trial showed increased mortality, transplant, and colorectal neoplasia; there is no proven medical therapy. Check serum IgG4 and add an IgG4 stain to the biopsy at every new diagnosis, and trial prednisone forty milligrams daily for four weeks then taper in the older man with painless jaundice, a distal common bile duct stricture, and a sausage-shaped pancreas rather than defaulting to a Whipple. For the full chapter with MCQs, tables, and primary-guideline references, visit www.boardpearls.com. Questions or feedback: [email protected]. (00:00) - The man with ulcerative colitis and rising ALP (00:34) - Presentation and the IBD association (02:07) - MRCP as the diagnostic test (04:27) - Natural history and premalignant surveillance (06:17) - The dominant stricture workup (08:07) - Gallbladder and colorectal thresholds (10:22) - No proven therapy and transplant (11:35) - IgG4-related sclerosing cholangitis (15:09) - The overlap syndromes

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