Primary Sclerosing Cholangitis Guidelines

Updated: Nov 21, 2019
  • Author: Vikas Khurana, MD, FACP, FACG; Chief Editor: Vinay Kumar Kapoor, MBBS, MS, FRCS, FAMS  more...
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Guidelines

Guidelines Summary

In August 2019, the British Society of Gastroenterology (BSG) and UK-PSC published guidelines for the prevention and treatment of primary sclerosing cholangitis. [39] These guidelines are summarized below.

Primary Sclerosing Cholangitis

Cholestatic liver biochemistry with typical cholangiographic features in the absence of other identifiable causes of secondary sclerosing cholangitis is usually sufficient for a diagnosis of primary sclerosing cholangitis (PSC).

Magnetic resonance cholangiopancreatography (MRCP) is recommended as the principal imaging modality for investigating suspected PSC.

Liver biopsy should be reserved for possible small duct PSC, assessment of suspected possible overlap variants, or when the diagnosis is unclear.

Risk stratification based on non-invasive assessment is recommended.

Do not use ursodeoxycholic acid (UDCA) for the prevention of colorectal cancer or cholangiocarcinoma.

Corticosteroids and immunosuppressants are not recommended for the treatment of classic PSC; however, corticosteroids may be indicated in patients with additional features of autoimmune hepatitis (AIH) or IgG4-related sclerosing cholangitis (IgG4-SC).

Perform endoscopic screening for esophageal varices in line with international guidelines where there is evidence of cirrhosis and/or portal hypertension.

Use colonoscopy and colonic biopsies to seek colitis in all patients with PSC.

Patients with suspected PSC undergoing endoscopic retrograde cholangiopancreatography (ERCP) should receive prophylactic antibiotics.

Non-invasive investigations such as MRCP, dynamic liver MRI, and/or contrast CT should be performed in patients who have new or changing symptoms or evolving abnormalities.

Patients with PSC should not undergo ERCP until there has been expert multidisciplinary assessment to justify endoscopic intervention.

In patients undergoing ERCP for dominant strictures, pathological sampling of suspicious strictures is mandatory.

Biliary dilatation is preferred to the insertion of biliary stents in patients undergoing ERCP for dominant strictures.

Assess eligibility and referral for liver transplantation in patients with PSC.

Assess all patients with PSC for osteoporosis.

When cholangiocarcinoma is suspected, use contrast-enhanced, cross-sectional imaging for diagnosis and staging.

Encourage patients with PSC to participate in support groups.

IgG4-related Sclerosing Cholangitis

Do not rely on elevated serum IgG4 levels to make a definitive diagnosis of IgG4-related disease (IgG4-RD) or distinguish it from PSC.

Obtain a confirmatory histological diagnosis in patients with suspected IgG4-related sclerosing cholangitis (IgG4-SC).

Recognize that other organ involvement (in particular, pancreatic manifestations of IgG4-RD) may provide important information to distinguish IgG4-SC from PSC.

Diagnose IgG4-SC according to recommendations of international consensus guidelines.

Corticosteroids should be given as first-line treatment.

Consider all patients with IgG4-SC, including those with multiorgan involvement in IgG4-RD, for continued immunosuppressive therapy.

Refer patients with complex IgG4-SC and those with suspected malignancy to a specialist.

Resources

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