Since people with primary sclerosing cholangitis may not have noticeable symptoms for many years, the disease often is suspected due to abnormal blood tests (taken for other reasons) that show a high level of liver enzymes (which indicate abnormal liver function). The disease also might be suspected due to a history of inflammatory bowel disease.
X-ray techniques can reveal whether bile ducts are blocked; however, they may not be able to determine the cause or site of the possible obstruction.
Sclerosing cholangitis is diagnosed by blood tests, along with radiologic imaging. Cholangiography is “taking a picture of the bile ducts.” The first choice for imaging is by injecting dye into the bile ducts and taking a magnetic resonance cholangiography (MRCP). The test can determine the cause and site of the blockage.
Many times a liver biopsy is also needed to confirm diagnosis and determine the progression of the disease.
Other tests that can be used to help with the diagnosis are:
- Endoscopic retrograde cholangiography (ERCP) in which dye is injected and a lighted and flexible endoscope (instrument used to visually examine the inside of certain body parts) is inserted through the mouth, stomach and then into the small intestine. A thin tube is placed through the scope and into the pancreatic and bile ducts, and the dye is injected to show the bile ducts on the X-ray.
- Percutaneous transhepatic cholangiography is when a dilated (enlarged) bile duct is found with an ultrasound, a needle punctures the skin and places dye directly into the bile duct and then X-rays are taken to identify blockage.
All of these procedures are done with the child sedated (given a medication that has a soothing and calming effect) or with anesthesia (medication given to put the child in a deep sleep with support to breathing available.)