Clinical features and management of primary sclerosing cholangitis.
Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, resulting in cirrhosis and need for liver transplantation and reduced life expectancy. The majority of cases occur in young and middle-aged men, often in association with inflammatory bowel disease. The etiology of primary sclerosing cholangitis includes immune-mediated components and elements of undefined nature. No effective medical therapy has been identified. The multiple complications of primary sclerosing cholangitis include metabolic bone disease, dominant strictures, bacterial cholangitis, and malignancy, particularly cholangiocarcinoma, which is the most lethal complication of primary sclerosing cholangitis. Liver transplantation is currently the only life-extending therapeutic alternative for patients with end-stage disease, although recurrence in the allografted liver has been described. A PSC-like variant attracting attention is cholangitis marked by raised levels of the immunoglobulin G4 subclass, prominence of plasma cells within the lesions, and steroid responsiveness.
Silveira
Marina-G
MG
Division of Gastroenterology and Hepatology, Miles and Shirley Fiterman Center for Digestive Diseases, Mayo Clinic and Foundation, Rochester, MN 55905, USA.
Lindor
Keith-D
KD
D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D001662:Biliary Tract Surgical Procedures; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D013505:Digestive System Surgical Procedures / Q000379:methods; D018450:Disease Progression; D004359:Drug Therapy, Combination; D015507:Drugs, Investigational / Q000627:therapeutic use; D016145:Endoscopy, Digestive System; D005260:Female; D005765:Gastrointestinal Agents / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016031:Liver Transplantation; D008297:Male; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis / Q000628:therapy; D012008:Recurrence; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D002756:Cholagogues and Choleretics; D015507:Drugs, Investigational; D005765:Gastrointestinal Agents; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid