Primary Sclerosing Cholangitis

Publication Date: May 1, 2015
Last Updated: March 14, 2022

RECOMMENDATIONS

Diagnosis

MRCP is preferred over ERCP to establish a diagnosis of PSC. (Strong “We recommend”, Moderate)
317606
Liver biopsy is not necessary to make a diagnosis in patients with suspected PSC based on diagnostic cholangiographics findings. (Conditional (weak) “We suggest”, Low)
317606
Liver biopsy is recommended to make a diagnosis in patients with suspected small duct PSC or to exclude other conditions such as suspected overlap with autoimmune hepatitis. (Conditional (weak) “We suggest”, Moderate)
317606
Antimitochondrial autoantibody testing can help exclude primary biliary cirrhosis. (Conditional (weak) “We suggest”, Moderate)
317606
Patients with PSC should be tested at least once for elevated serum IgG4 levels. (Conditional (weak) “We suggest”, Moderate)
317606

MEDICAL TREATMENT

UDCA in doses >28 mg/kg/day should not be used for management of patients with PSC. (Strong “We recommend”, High)
317606

ENDOSCOPIC MANAGEMENT

ERCP with balloon dilatation is recommended for PSC patients with dominant stricture and pruritus, and/or cholangitis, to relieve symptoms. (Strong “We recommend”, Low)
317606
PSC with a dominant stricture seen on imaging should have an ERCP with cytology, biopsies and FISH to exclude diagnosis of cholangiocarcinoma. (Strong “We recommend”, Low)
317606
PSC patients undergoing ERCP should have antibiotic prophylaxis to prevent post-ERCP cholangitis. (Conditional (weak) “We suggest”, Low)
317606
Routine stenting after dilation of a dominant stricture is not required, whereas short-term stenting may be required in patients with severe stricture. (Conditional (weak) “We suggest”, Low)
317606

LIVER TRANSPLANTATION

Liver transplantation, when possible, is recommended over medical therapy or surgical drainage in PSC patients with decompensated cirrhosis, to prolong survival. (Strong “We recommend”, Moderate)
317606
Patients should be referred for liver transplantation when their MELD score exceeds 14. (Conditional (weak) “We suggest”, Moderate)
317606

PSC AND IBD

Annual colon surveillance preferably with chromoendoscopy is recommended in PSC patients with colitis beginning at the time of PSC diagnosis. (Conditional (weak) “We suggest”, Moderate)
317606
A full colonoscopy with biopsies is recommended in patients with PSC regardless of the presence of symptoms to assess for associated colitis at the time of PSC diagnosis. (Conditional (weak) “We suggest”, Moderate)
317606
Some advocate repeating the exam every 3–5 years in those without prior evidence of colitis. (Conditional (weak) “We suggest”, Low)
317606

HEPATOBILIARY MALIGNANCIES AND GALLBLADDER DISEASE

Consider screening for cholangiocarcinoma with regular cross-sectional imaging with ultrasound or MR and serial CA 19-9 every 6–12 months. (Conditional (weak) “We suggest”, Very low)
317606
Cholecystectomy should be performed for patients with PSC and gallbladder polyps >8 mm, to prevent the development of gallbladder adenocarcinoma. (Conditional (weak) “We suggest”, Very low)
317606

SPECIAL SITUATIONS

Further testing for autoimmune hepatitis is recommended for patients <25 years of age with PSC or those with higher-than-expected levels of aminotransferases usually 5 × upper limit of normal. (Conditional (weak) “We suggest”, Moderate)
317606
MRCP is recommended for patients <25 years of age with autoimmune hepatitis, who have elevated serum ALP usually >2 × the upper limit of normal. (Conditional (weak) “We suggest”, Moderate)
317606

GENERAL MANAGEMENT

Local skin treatment should be performed with emollients and/or antihistamines in patients with PSC and mild pruritus, to reduce symptoms. (Conditional (weak) “We suggest”, Very low)
317606
Bile acid sequestrants such as cholestyramine should be taken (prescribed) in patients with PSC and moderate pruritus, to reduce symptoms. Second-line treatment such as rifampin and naltrexone can be considered if cholestyramine is ineffective or poorly tolerated. (Conditional (weak) “We suggest”, Very low)
317606
Recommend screening for varices in patients with signs of advanced disease with platelet counts <150 × 103/dl. (Conditional (weak) “We suggest”, Very low)
317606
Patients with PSC should undergo BMD screening at diagnosis with dual energy X-ray absorption at diagnosis and repeated at 2- to 4-year intervals. (Conditional (weak) “We suggest”, Moderate)
317606
Patients with advanced liver disease should be screened and monitored for fat-soluble vitamin deficiencies. (Conditional (weak) “We suggest”, Moderate)
317606

Recommendation Grading

Disclaimer

Overview

Title

Primary Sclerosing Cholangitis

Authoring Organization

Publication Month/Year

May 1, 2015

Last Updated Month/Year

June 27, 2023

Supplemental Implementation Tools

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Document Objectives

This Guideline for clinical practice is intended to indicate preferred approaches to Primary Sclerosing cholangitis as established by scientifically valid research. 

Target Patient Population

Patients with primary sclerosing cholangitis

Inclusion Criteria

Female, Male, Adolescent, Adult, Child, Older adult

Health Care Settings

Ambulatory, Hospital, Long term care, Outpatient

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Assessment and screening, Diagnosis, Management, Treatment

Diseases/Conditions (MeSH)

D002761 - Cholangitis, D015209 - Cholangitis, Sclerosing, D014580 - Ursodeoxycholic Acid

Keywords

cholangitis

Source Citation

Lindor, Keith D MD, FACG; Kowdley, Kris V MD, FACG; Harrison, Edwyn M MD. ACG Clinical Guideline: Primary Sclerosing Cholangitis, American Journal of Gastroenterology: May 2015 - Volume 110 - Issue 5 - p 646-659 doi: 10.1038/ajg.2015.112