Primary Sclerosing Cholangitis Clinical Presentation

  • Author: Vikas Khurana, MD, FACP, FACG; Chief Editor: Julian Katz, MD   more...
 
Updated: Apr 5, 2012
 

History

Approximately 75-90% of patients with primary sclerosing cholangitis (PSC) have inflammatory bowel disease (IBD). Of these, 87% have ulcerative colitis and 13% have Crohn colitis; however, the course of IBD is not related to PSC. Most patients are male and are diagnosed at a mean age of approximately 40 years.[8]

Symptoms upon initial presentation consist of fatigue, jaundice, pruritus, and right upper quadrant pain. The clinical course varies a great deal. Symptoms may remit and then recur spontaneously. Occasionally, patients with PSC have an acute hepatitis-like presentation. Recurrent febrile episodes of bacterial cholangitis occur in 10-15% of patients during the course of PSC. Pancreatic duct involvement in PSC is uncommon, and pancreatic exocrine insufficiency is not correlated when ductal abnormalities are noted.

Patients with asymptomatic PSC constitute 20-40% of the cohort in some large studies. This high percentage is thought to be attributable to the practices of screening patients with ulcerative colitis for elevated alkaline phosphatase levels and performing endoscopic retrograde cholangiopancreatography (ERCP). Cirrhosis, portal hypertension, and liver failure occur in progressive disease, with symptoms consistent with these entities, including variceal bleeding, ascites, and hepatic encephalopathy. The risk for cholangiocarcinoma is increased significantly in patients with PSC.[9, 10]

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Physical Examination

Physical examination results are significant for jaundice, weight loss, and, occasionally, pruritic skin marks. Hepatomegaly is common, and splenomegaly is present in up to one third of patients with PSC. As the disease progresses, signs of liver failure, including spider angiomata, ascites, and muscle atrophy, become apparent.

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Staging

Ludwig and associates described 4 stages of PSC,[11] as follows:

  • Stage 1: portal hepatitis, degeneration of bile ducts with inflammatory cell infiltrate
  • Stage 2: extension of disease to periportal area with prominent bile ductopenia
  • Stage 3: septal fibrosis and necrosis
  • Stage 4: frank cirrhosis
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Complications

Cholangitis and cholelithiasis occur more frequently in patients with primary sclerosing cholangitis (PSC). Chronic cholestasis leads to steatorrhea, fat-soluble vitamin deficiency (vitamins A, D, E, and K), metabolic bone disease with osteoporosis, and calorie loss with resultant weight loss. Secondary biliary cirrhosis due to chronic cholestasis occurs in patients with PSC. Portal hypertension with variceal bleeding, ascites, and liver failure then ensue.

Cholangiocarcinoma reportedly occurs in association with PSC in 6-30% of patients; on autopsy, it is found in up to 30-40% of patients with PSC. The development of cholangiocarcinoma remains unpredictable in any given patient, and no reliable serologic tumor markers have been identified. Worsening jaundice, pruritus, or weight loss may indicate development of a stricture or cholangiocarcinoma

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. [#FollowupPatientEducation] [#MiscellaneousMedicalLegalPitfalls]

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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, AGAF  Gastroenterologist, Ochsner Clinic Foundation; Adjunct Associate Research Scientist, Lovelace Respiratory Research Institute; Editor-in-Chief, The Internet Journal of Gasteroenterology; Editorial Board, Signal Transduction Insights; Editorial Board, The Internet Journal of Epidemiology; Editorial Board, Gastrointestinal Endoscopy Review Letter

Praveen K Roy, MD, AGAF is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, and American Society of Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

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 & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

Additional Contributors

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.

David Greenwald, MD Associate Professor of Clinical Medicine, Fellowship Program Director, 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, American Society for Gastrointestinal Endoscopy, and New York Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References

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Periductal onion skin fibrosis seen in primary sclerosing cholangitis.
Endoscopic retrograde cholangiopancreatography performed in a patient with abnormal liver function test results shows multiple intrahepatic bile duct strictures and beading.
Double-contrast barium enema (same patient as in the previous image) shows filiform polyps and an ahaustral colon resulting from ulcerative colitis.
Percutaneous transhepatic cholangiogram shows dilatation, stricturing, and beading of the intrahepatic bile ducts. Note the surgical clips from a previous cholecystectomy.
T-tube cholangiogram shows irregularity of the common bile duct, stricturing, beading, and dilatation of the intrahepatic bile ducts. Note a calculus in the termination of the left hepatic duct (arrow).
Magnetic resonance cholangiopancreatography shows a normal-sized common bile duct, but strictures of both the left and right ducts are noted as well as a dilated proximal left hepatic duct.
Technetium-99m iminodiacetic acid scan shows retention of the radionuclide proximal to strictures in the distribution of the left hepatic duct. Note the lack of filling of the gallbladder because of a previous cholecystectomy. Isotope has entered the small bowel.
 
 
 
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