Primary sclerosing cholangitis

Marina G. Silveira, Keith Lindor

Research output: Contribution to journalArticle

52 Citations (Scopus)

Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, resulting in end-stage liver disease and reduced life expectancy. PSC primarily affects young and middle-aged men, often in association with underlying inflammatory bowel disease. The etiology of PSC includes immune-mediated components and elements of undefined nature. A cholestatic picture of liver biochemistries with elevations in serum alkaline phosphatase, nonspecific autoantibodies such as perinuclear antineutrophilic antibody, antinuclear antibodies and smooth muscle antibodies, and diffuse multifocal biliary strictures, resulting in a 'beaded' appearance on radiographic studies, are the hallmarks of the disease. No effective medical therapy is currently available, although clinical studies are in progress. Ursodeoxycholic acid at high doses (28 mg/kg/day to 30 mg/kg/day) is the most promising agent but is unproven so far. Liver transplantation is currently the only life-extending therapy for patients with end-stage disease, although recurrent disease can be observed in the transplanted liver. The multiple complications of PSC include pruritus, fatigue, vitamin deficiencies, metabolic bone disease, peristomal varices, bacterial cholangitis, dominant biliary strictures, gallbladder stones and polyps, and malignancy, particularly cholangiocarcinoma, which is the most lethal complication of PSC.

Original languageEnglish (US)
Pages (from-to)689-698
Number of pages10
JournalCanadian Journal of Gastroenterology
Volume22
Issue number8
StatePublished - Aug 2008
Externally publishedYes

Fingerprint

Sclerosing Cholangitis
Pathologic Constriction
Avitaminosis
Ursodeoxycholic Acid
End Stage Liver Disease
Cholangitis
Cholangiocarcinoma
Antibodies
Metabolic Bone Diseases
Liver
Antinuclear Antibodies
Varicose Veins
Pruritus
Polyps
Life Expectancy
Bile Ducts
Gallbladder
Inflammatory Bowel Diseases
Biochemistry
Liver Transplantation

Keywords

  • Cholangiocarcinoma
  • Cholestasis
  • Diagnosis
  • Liver transplantation
  • Sclerosing cholangitis
  • Therapy

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Primary sclerosing cholangitis. / Silveira, Marina G.; Lindor, Keith.

In: Canadian Journal of Gastroenterology, Vol. 22, No. 8, 08.2008, p. 689-698.

Research output: Contribution to journalArticle

Silveira, MG & Lindor, K 2008, 'Primary sclerosing cholangitis', Canadian Journal of Gastroenterology, vol. 22, no. 8, pp. 689-698.
Silveira, Marina G. ; Lindor, Keith. / Primary sclerosing cholangitis. In: Canadian Journal of Gastroenterology. 2008 ; Vol. 22, No. 8. pp. 689-698.
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