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Guideline Summary
Guideline Title
Diagnosis and management of primary sclerosing cholangitis.
Bibliographic Source(s)
Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, Gores GJ, American Association for the Study of Liver Diseases. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010 Feb;51(2):660-78. [196 references] PubMed External Web Site Policy
Guideline Status

This is the current release of the guideline.

Scope

Disease/Condition(s)

Primary sclerosing cholangitis (PSC) and associated disorders, including:

  • Portal hypertension
  • Metabolic bone disease
  • Inflammatory bowel disease (IBD)
  • Gallbladder disease
  • Cholangiocarcinoma (CCA)
  • Biliary strictures
Guideline Category
Diagnosis
Evaluation
Management
Treatment
Clinical Specialty
Endocrinology
Family Practice
Gastroenterology
Internal Medicine
Obstetrics and Gynecology
Oncology
Pathology
Pediatrics
Surgery
Intended Users
Health Care Providers
Guideline Objective(s)

To provide clinicians with approaches to the diagnosis and management of primary sclerosing cholangitis

Target Population

Adults and children with primary sclerosing cholangitis

Interventions and Practices Considered

Primary Sclerosing Cholangitis (PSC) in Adults

Diagnosis/Evaluation

  1. History and assessment of signs and symptoms
  2. Serum biochemical tests
  3. Magnetic resonance cholangiography (MRC)
  4. Endoscopic retrograde cholangiography (ERC)
  5. Liver biopsy in selected patients
  6. Measuring serum immunoglobulin G4 (IgG4) levels

Management/Treatment

  1. Management of dominant strictures
    • Endoscopic dilatation with or without stenting
    • Biliary tract dilatation by percutaneous cholangiography with or without stenting if indicated
    • Surgical therapy in selected patients without cirrhosis
    • Antimicrobial therapy
    • Evaluation for liver transplantation
  2. Management of metabolic bone disease
    • Bone density examinations
    • Calcium and vitamin D
    • Bisphosphonate therapy (oral and parenteral)
  3. Management of inflammatory bowel disease (IBD) including colonoscopy and biopsy
  4. Management of gallbladder disease including annual ultrasound and cholecystectomy
  5. Management of cholangiocarcinoma (CCA) including surgical resection and consideration of liver transplantation
  6. Specific medical therapy including corticosteroids and other immunosuppressive agents
  7. Management of pregnancy in patients with PSC

Note: The use of prognostic models for predicting clinical outcomes was considered but not recommended. The use of ursodeoxycholic acid (UCDA) as chemoprevention for colorectal cancer was considered but not recommended.

Pediatric PSC

  1. Liver biopsy
  2. Immunosuppressive agents
  3. Liver transplantation

Note: The use of screening and surveillance procedures for detecting biliary tract cancer in children with PSC was considered but not recommended.

Major Outcomes Considered
  • Sensitivity and specificity of diagnostic tests
  • Prevalence of dominant strictures, inflammatory bowel disease (IBD), gallbladder disease, and cholangiocarcinoma (CCA) in patients with primary sclerosing cholangitis (PSC)
  • Effectiveness of treatment
  • Survival rates

Methodology

Methods Used to Collect/Select the Evidence
Hand-searches of Published Literature (Primary Sources)
Hand-searches of Published Literature (Secondary Sources)
Searches of Electronic Databases
Description of Methods Used to Collect/Select the Evidence

The recommendations are based on the following: (1) formal review and analysis of the recently published world literature on the topic (Medline search); (2) American College of Physicians Manual for Assessing Health Practices and Designing Practice Guidelines; (3) guideline policies, including the American Association for the Study of Liver Diseases (AASLD) Policy on the Development and Use of Practice Guidelines and the American Gastroenterological Association Policy Statement on Guidelines; and (4) the experience of the authors in the specified topic.

Number of Source Documents

Not stated

Methods Used to Assess the Quality and Strength of the Evidence
Weighting According to a Rating Scheme (Scheme Given)
Rating Scheme for the Strength of the Evidence

Quality of Evidence

High (A) - Further research is unlikely to change confidence in the estimate of the clinical effect.

Moderate (B) - Further research may change confidence in the estimate of the clinical effect.

Low (C) - Further research is very likely to impact confidence on the estimate of clinical effect.

Methods Used to Analyze the Evidence
Review of Published Meta-Analyses
Systematic Review
Description of the Methods Used to Analyze the Evidence

Not stated

Methods Used to Formulate the Recommendations
Expert Consensus
Description of Methods Used to Formulate the Recommendations

Not stated

Rating Scheme for the Strength of the Recommendations

Strength of Recommendation

Strong (1) - Factors influencing the strength of the recommendation included the quality of the evidence, presumed patient-important outcomes, and cost.

Weak (2) - Variability in preferences and values, or more uncertainty. Recommendation is made with less certainty, higher cost or resource consumption.

Cost Analysis

A formal cost analysis was not performed and published cost analyses were not reviewed.

Method of Guideline Validation
Peer Review
Description of Method of Guideline Validation

This guideline has been approved by the American Association for the Study of Liver Diseases (AASLD) and represents the position of the association. This practice guideline was produced in collaboration with the Practice Guidelines Committee of the AASLD, which provided extensive peer review of the manuscript.

Recommendations

Major Recommendations

The strength of recommendation (Strong [1] and Weak [2]) and the quality of evidence (A–C) are defined at the end of the "Major Recommendations."

Definition and Diagnosis

  1. In patients with cholestatic biochemical profile, the guideline developers recommend indirect magnetic resonance cholangiography (MRC) or direct endoscopic retrograde cholangiopancreatography (ERCP) for making the diagnosis of primary sclerosing cholangitis (PSC) (1A).
  2. The guideline developers recommend against routine liver biopsy for the diagnosis of PSC in patients with typical cholangiographic findings (1B).
  3. In patients with a normal endoscopic retrograde cholangiography (ERC) or MRC, a liver biopsy is recommended to diagnose small duct PSC (1B).
  4. In patients with disproportionately elevated aminotransferases, a liver biopsy is recommended to diagnose or exclude overlap syndrome (1B).
  5. In all patients with possible PSC, the guideline developers suggest measuring serum immunoglobulin G (IgG)4 levels to exclude IgG4-associated sclerosing cholangitis (2C).

Dominant Strictures in PSC

  1. In patients with increases in serum bilirubin and/or worsening pruritus, progressive bile duct dilatation on imaging studies, and/or cholangitis, the guideline developers recommend performing an ERC promptly to exclude a dominant stricture (1B).
  2. In patients with dominant strictures from PSC, initial management with endoscopic dilatation with or without stenting is recommended (1B).
  3. In patients with dominant strictures from PSC in whom an endoscopic approach is unsuccessful, biliary tract dilatation by percutaneous cholangiography with or without stenting should be considered (1B).
  4. Brush cytology and/or endoscopic biopsy is recommended to exclude a superimposed malignancy prior to endoscopic therapy for dominant strictures (1B).
  5. In patients with dominant strictures refractory to endoscopic and/or percutaneous management, surgical therapy in selected patients without cirrhosis C is recommended (1B).
  6. The guideline developers recommend antimicrobial therapy with correction of bile duct obstruction in dominant strictures to effectively resolve cholangitis (1A).
  7. In patients with recurrent bacterial cholangitis, the use of prophylactic long-term antibiotics is recommended (1B).
  8. In patients with refractory bacterial cholangitis, evaluation for liver transplantation is recommended (1B).

Metabolic Bone Disease

  1. Bone density examinations are recommended to exclude osteopenia or osteoporosis at diagnosis and, thereafter, at 2-3 year intervals (1B).
  2. In patients with hepatic osteopenia, the guideline developers suggest the use of calcium 1.0 to 1.5 g and vitamin D 1,000 IU daily for therapy (2C).
  3. In patients with hepatic osteoporosis, the guideline developers suggest the use of bisphosphonate therapy in addition to calcium and vitamin D supplementation (2C).
  4. In patients with osteoporosis and esophageal varices, the use of parenteral forms of bisphosphonate therapy rather than oral formulations is suggested (2C).

Inflammatory Bowel Disease (IBD) and PSC

  1. The guideline developers recommend full colonoscopy with biopsies in patients with a new diagnosis of PSC and no previous history or symptoms of IBD (1A).
  2. In patients with IBD and PSC, surveillance colonoscopy with biopsies is recommended at 1-year to 2-year intervals from the time of diagnosis of PSC to exclude colorectal neoplasia (1B).
  3. The guideline developers recommend against the use of ursodeoxycholic acid (UDCA) as chemoprevention for colorectal cancer in patients with ulcerative colitis and PSC (1B).
  4. The guideline developers recommend that patients with IBD and PSC should be treated according to guidelines for IBD (1B).

Gallbladder Disease and PSC

  1. The guideline developers recommend annual ultrasound to detect mass lesions in the gallbladder (1C).
  2. In patients with gallbladder mass lesions, cholecystectomy is recommended as treatment regardless of lesion size, if the underlying liver disease permits (1C).

Cholangiocarcinoma (CCA)

  1. The guideline developers recommend evaluation for CCA in patients with deterioration of their constitutional performance status or liver biochemical-related parameters (1B).
  2. In patients with CCA and the absence of cirrhosis, the guideline developers suggest that surgical resection may be performed (2B).
  3. In patients with early stage CCA not amenable to surgical resection, the guideline developers recommend that such patients be considered for liver transplantation following neoadjuvant therapy by experienced transplant centers (1B).

Natural History and Prognostic Models

  1. In patients with PSC, the guideline developers recommend against the use of prognostic models for predicting clinical outcomes in an individual patient as no consensus exists regarding the optimal model (1B).

Specific Medical Therapy

  1. In adult patients with PSC, the guideline developers recommend against the use of UDCA as medical therapy (1A).
  2. In adult patients with PSC and overlap syndrome, the use of corticosteroids and other immunosuppressive agents are recommended for medical therapy (1C).

Liver Transplantation

  1. In patients with advanced liver disease, the use of liver transplantation is recommended as a successful treatment modality (1A).
  2. The guideline developers recommend excluding alternate causes of biliary strictures in the posttransplant setting before making a diagnosis of recurrent PSC (1B).

Fertility and Pregnancy in PSC

  1. In female patients of childbearing age without portal hypertension, the guideline developers recommend that pregnancy can be completed safely under close medical supervision (1C).

Pediatric PSC

  1. In children liver biopsy should be used to diagnose overlap syndrome with PSC and autoimmune hepatitis (1B).
  2. In children with overlap syndrome, the use of immunosuppressive agents is recommended for medical therapy (1B).
  3. The guideline developers recommend against the use of screening and surveillance procedures for detecting biliary tract cancer in children with PSC; approaches to colorectal cancer screening in children with IBD should not be influenced by the diagnosis of PSC (1B).
  4. In children with end-stage liver disease from PSC, the use of liver transplantation is recommended as effective therapy (1A).

Definitions:

Quality of Evidence

High (A) - Further research is unlikely to change confidence in the estimate of the clinical effect.

Moderate (B) - Further research may change confidence in the estimate of the clinical effect.

Low (C) - Further research is very likely to impact confidence on the estimate of clinical effect.

Strength of Recommendation

Strong (1) - Factors influencing the strength of the recommendation included the quality of the evidence, presumed patient-important outcomes, and cost.

Weak (2) - Variability in preferences and values, or more uncertainty. Recommendation is made with less certainty, higher cost or resource consumption.

Clinical Algorithm(s)

Clinical algorithms are provided in the original guideline for:

  • Diagnosis of primary sclerosing cholangitis (PSC)
  • Diagnostic evaluation of a dominant stricture suspicious for cholangiocarcinoma (CCA)

Evidence Supporting the Recommendations

Type of Evidence Supporting the Recommendations

The type of supporting evidence is identified and graded for each recommendation (see "Major Recommendations").

Benefits/Harms of Implementing the Guideline Recommendations

Potential Benefits

Appropriate diagnosis and management of primary sclerosing cholangitis

Potential Harms
  • Endoscopic retrograde cholangiography (ERC) is an invasive procedure associated with potentially serious complications such as pancreatitis and bacterial cholangitis. Indeed, ERC is associated with hospitalization in up to 10% of primary sclerosing cholangitis (PSC) patients undergoing the procedure.
  • It should be noted that early changes of PSC may be missed by magnetic resonance cholangiography (MRC).
  • Biliary stenting has been shown to be associated with increased complications when compared to endoscopic dilatation only and should be reserved for strictures that are refractory to dilatation.
  • All therapeutic endoscopy comes with risk. In the two largest reported series of patients with long follow-up, the risk of complications was 7.3% to 20%. The complications were mild without need for surgical intervention. The most common complications were pancreatitis, cholangitis, biliary tract perforation and hemorrhage.
  • The percutaneous management approach is associated with increased morbidity but similar efficacy as the endoscopic approach and is reserved for patients who have proximal dominant strictures with a failed endoscopic approach.

Qualifying Statements

Qualifying Statements

Intended for use by physicians, these recommendations suggest preferred approaches to the diagnostic, therapeutic and preventative aspects of care. They are intended to be flexible, in contrast to standards of care, which are inflexible policies to be followed in every case.

Implementation of the Guideline

Description of Implementation Strategy

An implementation strategy was not provided.

Implementation Tools
Clinical Algorithm
For information about availability, see the Availability of Companion Documents and Patient Resources fields below.

Institute of Medicine (IOM) National Healthcare Quality Report Categories

IOM Care Need
Living with Illness
IOM Domain
Effectiveness

Identifying Information and Availability

Bibliographic Source(s)
Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, Gores GJ, American Association for the Study of Liver Diseases. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010 Feb;51(2):660-78. [196 references] PubMed External Web Site Policy
Adaptation

Not applicable: The guideline was not adapted from another source.

Date Released
2010 Feb
Guideline Developer(s)
American Association for the Study of Liver Diseases - Nonprofit Research Organization
Source(s) of Funding

American Association for the Study of Liver Diseases

Guideline Committee

Practice Guidelines Committee of the American Association for the Study of Liver Diseases

Composition of Group That Authored the Guideline

Primary Authors: Roger Chapman; Johan Fevery; Anthony Kalloo; David M. Nagorney; Kirsten Muri Boberg; Benjamin Shneider; Gregory J. Gores

Committee Members: Jayant A. Talwalkar, M.D., M.P.H. (Chair); Anna Mae Diehl, M.D. (Board Liaison); Jeffrey H. Albrecht, M.D.; Amanda DeVoss, M.M.S., P.A.-C.; José Franco, M.D.; Stephen A. Harrison, M.D.; Kevin Korenblat, M.D.; Simon C. Ling, M.B.Ch.B.; Lawrence U. Liu, M.D.; Paul Martin, M.D.; Kim M. Olthoff, M.D.; Robert S. O'Shea, M.D.; Nancy Reau, M.D.; Adnan Said, M.D.; Margaret C. Shuhart, M.D., M.S.; and Kerry N. Whitt, M.D.

Financial Disclosures/Conflicts of Interest

Potential conflict of interest: Nothing to report.

Guideline Status

This is the current release of the guideline.

Guideline Availability

Electronic copies: Available in Portable Document Format (PDF) from the American Association for the Study of Liver Diseases Web site External Web Site Policy.

Print copies: Available from the American Association for the Study of Liver Diseases, 1729 King Street, Suite 200; Alexandria, VA 22314; Phone: 703-299-9766; Web site: www.aasld.org External Web Site Policy; e-mail: aasld@aasld.org.

Availability of Companion Documents

None available

Patient Resources

None available

NGC Status

This summary was completed by ECRI Institute on August 13, 2010. The information was verified by the guideline developer on September 17, 2010. This summary was updated by ECRI Institute on December 10, 2010 following the U.S. Food and Drug Administration (FDA) advisory on Bisphosphonates.

Copyright Statement

This NGC summary is based on the original guideline, which is subject to the American Association for the Study of Liver Diseases' copyright restrictions.

Disclaimer

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