eMedicine Specialties > Gastroenterology > Biliary

Primary Sclerosing Cholangitis: Follow-up

Author: Vikas Khurana, MD, FACP, FACG, Assistant Professor, Department of Medicine, Division of Gastroenterology and Hepatology, Graduate Hospital, Gastroenterology Associates, PC
Coauthor(s): Tejinder Singh, MD, Lead Physician, Section of Emergency Services, Overton Brooks Veterans Affairs Medical Center; Praveen K Roy, MD, Comments and Criticisms Editor, Cochrane Colorectal Cancer Group
Contributor Information and Disclosures

Updated: Aug 28, 2009

Follow-up

Further Inpatient Care

  • Further inpatient care is dictated by the admitting diagnosis and complications and may involve any or all of the previously discussed treatment options.

Further Outpatient Care

  • Further outpatient care is dictated by the stage and degree of progression of the disease. It involves care by a gastroenterologist, who must monitor the patient's condition with frequent laboratory tests and maintain vigilance for the many complications of PSC. Suitability for liver transplantation should be determined expeditiously and, if appropriate, offered to the patient.

Deterrence/Prevention

  • PSC is a progressive disease, and no preventive strategies have been proven effective. Stopping smoking and/or drinking may decrease or delay the chances of developing cholangiocarcinoma.

Complications

  • Chronic cholestasis leads to steatorrhea, fat-soluble vitamin (A, D, E, and K) deficiency, metabolic bone disease with osteoporosis, and calorie loss with resultant weight loss.
  • Cholangitis and cholelithiasis occur more frequently in patients with PSC.
  • Cholangiocarcinoma reportedly occurs in association with PSC in 6-30% of patients and in up to 30-40% of patients with PSC on autopsy. The development of cholangiocarcinoma remains unpredictable in any given patient, and no reliable serological tumor markers have been identified.
  • Secondary biliary cirrhosis due to chronic cholestasis occurs in patients with PSC. Portal hypertension with variceal bleeding, ascites, and liver failure then ensue.
  • Dominant biliary strictures can be identified in about 20% of patients with PSC and must be differentiated from cholangiocarcinoma. Strictures cause cholestasis with jaundice and pruritus and may also result in cholangitis.
  • The risk of colon cancer is increased for patients with both ulcerative colitis and PSC versus those with only ulcerative colitis.

Prognosis

  • PSC is a progressive disorder that ultimately leads to hepatic failure. Median survival time without liver transplantation is about 12 years. Survival prospects are more dismal for those who are symptomatic at diagnosis. The following variables influence prognosis:
    • Revised Mayo Clinic model
      • Age
      • Serum bilirubin, albumin, and aspartate aminotransferase levels
      • Variceal bleeding history
    • Child-Turcotte-Pugh scale
      • Grade of encephalopathy
      • Presence or absence of ascites
      • Serum albumin level
      • Prothrombin time
      • Bilirubin level

Patient Education

  • Educate patients regarding the natural course of the disease, expected complications, and treatment strategies, including liver transplantation.

Miscellaneous

Medicolegal Pitfalls

  • Failure to conscientiously monitor and treat complications, including those caused by nutritional deficits (eg, night blindness, osteoporosis with resultant fractures)
  • Failure to recognize that worsening jaundice, pruritus, or weight loss may indicate development of a stricture or cholangiocarcinoma
  • Failure to perform colonoscopy or flexible sigmoidoscopy in patients with PSC without a history of IBD
  • Failure to conduct periodic colonoscopic surveillance of patients with PSC and ulcerative colitis to exclude colonic neoplasia
  • Failure to monitor for toxicity in patients taking certain immunosuppressant drugs
 


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
References
Further Reading
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References

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Further Reading

Related eMedicine Topics

Clinical Trials
 National Guideline Clearinghouse

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Keywords

primary sclerosing cholangitis, PSC, sclerosis of the liver, chronic liver disease, biliary cirrhosis, chronic hepatic disease, cholestasis, intrahepatic bile ducts, extrahepatic bile ducts, liver cirrhosis, portal hypertension, liver transplantation, liver transplant, inflammatory bowel disease, IBD,

ulcerative colitis, Crohn colitis, Crohn disease, bacterial cholangitis, cholangiocarcinoma, hepatomegaly, splenomegaly, endoscopic retrograde cholangiopancreatography, ERCP, magnetic resonance cholangiopancreatography, MRCP

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Contributor Information and Disclosures

Author

Vikas Khurana, MD, FACP, FACG, Assistant Professor, Department of Medicine, Division of Gastroenterology and Hepatology, Graduate Hospital, Gastroenterology Associates, PC
Disclosure: Nothing to disclose.

Coauthor(s)

Tejinder Singh, MD, Lead Physician, Section of Emergency Services, Overton Brooks Veterans Affairs Medical Center
Disclosure: Nothing to disclose.

Praveen K Roy, MD, Comments and Criticisms Editor, Cochrane Colorectal Cancer Group
Praveen K Roy, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, and Canadian Association of Gastroenterology
Disclosure: Nothing to disclose.

Medical Editor

David Greenwald, MD, Fellowship Program Director, Associate Professor, Department of Medicine, Division of Gastroenterology, Montefiore Medical Center, Albert Einstein College of Medicine
David Greenwald, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Simmy Bank, MD, Chair, Professor, Department of Internal Medicine, Division of Gastroenterology, Long Island Jewish Hospital, Albert Einstein College of Medicine
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law Medicine and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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