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Genetic and Rare Diseases Information Center (GARD)

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Primary sclerosing cholangitis

Other Names for this Disease
  • Cholangitis, primary sclerosing
  • Sclerosing cholangitis
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Your Question

What causes the pain in primary sclerosing cholangitis? Does increased pain indicate a worsening of the disease and thus the need to re-do tests?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

Does worsening pain indicate the progession of primary sclerosing cholangitis?

The natural course of primary sclerosing cholangitis (PSC) is chronic (long lasting) and progressive (worsens over time). Stomach pain can be an early symptom of PSC (e.g., associated with infection or gallstones), a symptom of progressed disease (e.g. cirrhosis, portal hypertension, cholestasis), or associated with a co-occurring autoimmune disorder (e.g., ulcerative colitis) or cancer (e.g., cholangiocarcinoma).   In addition to stomach pain, other signs and symptoms that could signal advanced disease or cancer include persistent jaundice and weight loss.[1] We strongly encourage you to speak with your healthcare provider regarding the cause of your increased pain.
Last updated: 3/4/2013

How often should screenings and tests be done to detect the progression and secondary complications of primary sclerosing cholangitis?

The signs and symptoms experienced by a person with primary sclerosing cholangitis (PSC) can help guide decisions regarding testing. The benefits of routine screening of people with PSC for early detection of cholangiocarcinoma is debated, however it has been suggested that people with PSC undergo annual screening for cholangiocarcinoma with either an ultrasound or MRI with MRCP, plus measurement of a serum CA 19-9.[1] Once again, we recommend that you speak with your healthcare provider to learn more about your testing and screening options.
Last updated: 3/4/2013

  • Kowdley KV. Treatment of Primary Sclerosing Cholangitis. In: Basow, DS (Ed). UpToDate. Waltham, MA: UpToDate; 2012;
  • Kowdley KV. Clinical Manifestations and Diagnosis of Primary Sclerosing Cholangitis. In: Basow, DS (Ed). UpToDate. Waltham, MA: UpToDate; 2012;