Current Concepts in Primary Sclerosing Cholangitis

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Current Concepts in Primary Sclerosing Cholangitis RUSSELL H. WIESNER, M.D.  Mayo Clinic Proceedings  Volume 69, Issue 10, Pages 969-982 (October 1994) DOI: 10.1016/S0025-6196(12)61822-9 Copyright © 1994 Mayo Foundation for Medical Education and Research Terms and Conditions

Fig. 1 Endoscopic retrograde cholangiogram, showing classic findings in setting of primary sclerosing cholangitis, including strictures, beading, and irregularities of extrahepatic and intrahepatic biliary system. Mayo Clinic Proceedings 1994 69, 969-982DOI: (10.1016/S0025-6196(12)61822-9) Copyright © 1994 Mayo Foundation for Medical Education and Research Terms and Conditions

Fig. 2 Photomicrographs of liver biopsy specimens. A, Concentric peribiliary fibrosis and inflammation characteristic of early bile duct damage in setting of primary sclerosing cholangitis. (Hematoxylin-eosin; original magnification, ×300.) B, Concentric peribiliary fibrosis that leads to early obliteration of intralobular bile duct during late stages of primary sclerosing cholangitis. (Hematoxylin-eosin; original magnification, ×250.) Mayo Clinic Proceedings 1994 69, 969-982DOI: (10.1016/S0025-6196(12)61822-9) Copyright © 1994 Mayo Foundation for Medical Education and Research Terms and Conditions

Fig. 3 Kaplan-Meier estimated survival curves for asymptomatic and symptomatic patients with primary sclerosing cholangitis. For comparison, survival curves in US population matched for age, sex, and race to asymptomatic patients are also shown. Difference in survival: asymptomatic versus control subjects, P<0.001; symptomatic versus asymptomatic patients, P<0.003. (From Wiesner and associates.6 By permission of Mosby-Year Book.) Mayo Clinic Proceedings 1994 69, 969-982DOI: (10.1016/S0025-6196(12)61822-9) Copyright © 1994 Mayo Foundation for Medical Education and Research Terms and Conditions

Fig. 4 Graph depicting histopathologic evolution of primary sclerosing cholangitis from reversible stage, during which early bile duct damage occurs and which should respond to medical therapy, to irreversible fibrotic-cirrhotic stage, which is resistant to medical intervention. Mayo Clinic Proceedings 1994 69, 969-982DOI: (10.1016/S0025-6196(12)61822-9) Copyright © 1994 Mayo Foundation for Medical Education and Research Terms and Conditions

Fig. 5 Estimated survival (based on Kaplan-Meier analysis) after liver transplantation in patients with primary sclerosing cholangitis (PSC) and estimated survival without liver transplantation as predicted by Mayo Clinic survival model. Liver transplantation increases survival significantly in patients with end-stage PSC. (Modified from Abu-Elmagd and associates.73 By permission of Surgery, Gynecology & Obstetrics.) Mayo Clinic Proceedings 1994 69, 969-982DOI: (10.1016/S0025-6196(12)61822-9) Copyright © 1994 Mayo Foundation for Medical Education and Research Terms and Conditions

Fig. 6 Peristomal varices surrounding abdominal ileal stoma in patient with primary sclerosing cholangitis who had undergone proctocolectomy and Brooke ileostomy. Patient had daily bleeding from varices before undergoing liver transplantation. Mayo Clinic Proceedings 1994 69, 969-982DOI: (10.1016/S0025-6196(12)61822-9) Copyright © 1994 Mayo Foundation for Medical Education and Research Terms and Conditions

Fig. 7 Survival in patients undergoing liver transplantation for primary sclerosing cholangitis (PSC) in comparison with overall survival for all indications of orthotopic liver transplantation (OLTx). Patients undergoing liver transplantation for PSC had significantly improved survival (P<0.01) on the basis of 1992 data from United Network for Organ Sharing (UNOS). Patients with PSC, N = 499; other indications, N = 4,332. (From Wiesner RH. Managing complications of PSC and monitoring disease progression. Contemp Intern Med 1994 May; 6:37–46. By permission of Aegean Communications.) Mayo Clinic Proceedings 1994 69, 969-982DOI: (10.1016/S0025-6196(12)61822-9) Copyright © 1994 Mayo Foundation for Medical Education and Research Terms and Conditions