Primary Sclerosing Cholangitis

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Disease overview

PSC is an immune-mediated inflammatory disease causing fibrosis of intra- and extra-hepatic bile ducts [more]. Many patients have co-occurring inflammatory bowel disease, and progression to liver cirrhosis is common [more].

Common symptoms

Fatigue and pruritus are common initial signsjaundice and ascites indicate progressive liver damage. Patients may have hepatosplenomegaly and RUQ pain [ ]. Elevated alkaline phosphatase is very commonGGT elevation is also common but aminotransferases are only mildly elevated [ ] [ ].

WHEN TO SUSPECT

  • Recommendation 1

    U/S of the RUQ is recommended to rule out biliary obstruction

  • Recommendation 2

    if no obstruction, MRCP is the preferred initial imaging modality for diagnosis of PSC [more]. Liver biopsy can be confirmatory but is rarely required unless MRCP is unsuccessful in establishing diagnosis. All patients with PSC should be tested for IgG4 autoantibodies.

HOW TO TEST

  • Recommendation 1

    U/S of the RUQ is recommended to rule out biliary obstruction

  • Recommendation 2

    if no obstruction, MRCP is the preferred initial imaging modality for diagnosis of PSC [more]. Liver biopsy can be confirmatory but is rarely required unless MRCP is unsuccessful in establishing diagnosis. All patients with PSC should be tested for IgG4 autoantibodies.

TREATMENT

  • Recommendation 1

    Ursodeoxycholic acid therapy ranging from 3-12 months, avoiding doses above 20mg/kg/day, is the current standard of care, though clinical trials have shown mixed/controversial results [more]. Immunosuppressive therapies are under study. Many patients will progress to end-stage liver disease and require transplant. For dominant strictures felt to be symptomatic, balloon dilation is preferred to stenting [more]. Patients with IgG4+ disease may have a more rapid course that is less steroid-responsive [more].

PRIMARY CARE

  • Recommendation 1

    Patients will need frequent monitoring and likely consideration for liver transplant with disease progression

  • Recommendation 2

    patients are also at high risk for cholangiocarcinoma [more]. If a prior diagnosis of IBD has not been made, colonoscopy may be indicated given the frequent overlap between PSC and IBD [more].

FURTHER SUPPORT

  • Recommendation 1

    PSC Partners is a patient support organization maintaining a map of providers with PSC experience, including hepatologists and gastroenterologists. Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health.

CLINICAL TRIALS

  • Recommendation 1

    A large number of trials are currently recruiting in multiple countries.

Sources

No data available

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