eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Primary Sclerosing Cholangitis: Follow-up

Author: Lynette Gillis, MD, Assistant Professor, Director of Pediatric Hepatology and Liver Transplant, Division of Pediatric Gastroenterology, Hepatology and Nutrition and Division of Medical Genetics, Vanderbilt University
Contributor Information and Disclosures

Updated: Jun 19, 2009

Follow-up

Complications

  • Cholangiocarcinoma (CCA) develops in 10-15% of adult patients with primary sclerosing cholangitis (PSC).
    • Early detection of CCA is limited by a lack of reliable serologic, radiologic, and endoscopic findings.
    • CCA is often cholangiographically indistinguishable from a benign dominant stricture.
    • Brush cytology of dominant strictures has not shown predictive value.
    • Serum CA 19-9 recently appears useful (75% sensitivity, 80% specificity) in discriminating which patients with primary sclerosing cholangitis have CCA.
    • Indices of disease severity, such as the Mayo risk or Child-Pugh score, do not correlate with the likelihood of CCA development in patients with primary sclerosing cholangitis.
    • Risk factors are poorly understood, but alcohol consumption has been shown to be an independent risk factor for development of CCA in patients with primary sclerosing cholangitis. An association between previous or current smoking status and CCA has also been suggested.
  • The risk of colorectal cancer or dysplasia is increased in patients with ulcerative colitis (UC) and primary sclerosing cholangitis.
    • Chronically active disease may be a risk factor, whereas folate may have a protective effect.
    • Colorectal cancers associated with primary sclerosing cholangitis are more likely to be proximal, diagnosed at a more advanced stage, and fatal.
    • Colectomy in patients with UC and primary sclerosing cholangitis does not alter the natural history of primary sclerosing cholangitis.
  • Patients who have undergone transplantation are susceptible to a wide array of complications secondary to chronic immunosuppression.
    • The incidence of acute cellular and chronic ductopenic rejection is higher in patients with primary sclerosing cholangitis than in individuals of a non–primary sclerosing cholangitis control group.
    • Chronic ductopenic rejection adversely affects patient and graft survival.
    • Biliary strictures, both anastomotic and nonanastomotic, can occur.
    • Recurrent sclerosing cholangitis occurs in 10-20% of patients with primary sclerosing cholangitis who have undergone transplantation.
    • Data from the Mayo Clinic's review of 150 consecutive patients with primary sclerosing cholangitis who received 174 liver allografts suggests that postoperative biliary strictures or recurrence of primary sclerosing cholangitis does not impact patient survival.

Prognosis

  • Primary sclerosing cholangitis is characterized by a slow insidious progression to cirrhosis. In adult patients, the median period of survival from the time of diagnosis is 9-11 years. The median period of survival is shorter for patients who are symptomatic at the time of diagnosis.
  • The identification of abnormal liver function tests (LFTs) in patients with inflammatory bowel disease (IBD) has led to earlier diagnosis of primary sclerosing cholangitis, with apparent survival times that are likely longer.
  • Despite progress in early recognition, optimal treatment of patients with primary sclerosing cholangitis remains a challenge, requiring a multidisciplinary approach among hepatologists, endoscopists, surgeons, and interventional radiologists.
  • The coexistence of UC is not predictive of an increased risk of death in primary sclerosing cholangitis. UC may be associated with an increased posttransplantation survival.
 


More on Primary Sclerosing Cholangitis

Overview: Primary Sclerosing Cholangitis
Differential Diagnoses & Workup: Primary Sclerosing Cholangitis
Treatment & Medication: Primary Sclerosing Cholangitis
Follow-up: Primary Sclerosing Cholangitis
Multimedia: Primary Sclerosing Cholangitis
References
Further Reading
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References

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Keywords

primary sclerosing cholangitis, PSC, liver disease, cirrhosis, portal hypertension, liver failure, end-stage liver disease, hepatomegaly, liver transplantation, inflammatory bowel disease, IBD, ulcerative colitis, UC, hepatomegaly, cholestasis, pruritus, cholangitis, fat malabsorption, treatment, diagnosis

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Contributor Information and Disclosures

Author

Lynette Gillis, MD, Assistant Professor, Director of Pediatric Hepatology and Liver Transplant, Division of Pediatric Gastroenterology, Hepatology and Nutrition and Division of Medical Genetics, Vanderbilt University
Lynette Gillis, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Liver Foundation, and North American Society for Pediatric Gastroenterology, Hepatology and Nutrition
Disclosure: Nothing to disclose.

Medical Editor

Robert Baldassano, MD, Director, Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology and Nutrition, Associate Professor, Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania
Robert Baldassano, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

David A Piccoli, MD, Chief, Division of Gastroenterology and Nutrition, Department of Pediatrics, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine
David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

CME Editor

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center
Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research
Disclosure: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor

Chief Editor

Carmen Cuffari, MD, Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine
Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

 
 
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