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How is primary sclerosing cholangitis diagnosed?

Primary sclerosing cholangitis (PSC) is a chronic (lasting years and decades), progressive (worsening over time) disease of the bile ducts.
– This disease channelizes bile from the liver into the intestines.
– Bile is a watery liquid made by the cells of the liver that is important for digesting food in the intestine, particularly fat.
– This liquid also helps to get rid of the body of toxins.
– Liver cells secrete the bile they make into small canals within the liver.
– The bile flows through the canals and into larger collecting canals (ducts).
– The bile then flows within the intrahepatic bile ducts out of the liver and into the extrahepatic bile ducts.
– From the extrahepatic bile ducts, the bile flows into the intestine where the bile mixes with food.

In primary sclerosing cholangitis :
– The intrahepatic and extrahepatic bile ducts become inflamed,
– scarred and thickened (sclerotic),
– narrowed,
– finally obstructed.

Obstruction of the ducts can lead to:
– abdominal pain,
– itching,
– jaundice,
– infection in the bile ducts (cholangitis),
– liver scarring that leads to liver cirrhosis,
– liver failure.

The diagnosis of primary sclerosing cholangitis inclde:
– It is suspected from the symptoms (such as fatigue, itching, and jaundice).
– Abnormal laboratory tests (such as abnormally elevated blood levels of alkaline phosphatase and other liver enzymes).
– It is confirmed by demonstration of abnormally thickened bile ducts using special radiological tests.

It is also important to exclude diseases that can mimic primary sclerosing cholangitis like:
– primary biliary cirrhosis(PBC)
– gallstones in the bile ducts
– bile duct cancers
– strictures

Blood Tests
– The blood level of alkaline phosphates is usually elevated in primary sclerosing cholangitis.
– The blood levels of other liver enzymes (AST and ALT) may also be mildly elevated.
– Except in those patients with the autoimmune form of primary sclerosing cholangitis, the bilirubin usually is normal.
– It gradually increases as the disease progress.
– Antimitochondrial antibody (AMA), which is elevated in patients with PBC, is usually normal in these patients.

Radiologic Tests
– Endoscopic retrograde cholangio-pancreatography (ERCP).
– Magnetic resonance cholangio-pancreatography (MRCP).
– These are commonly performed to visualize the intrahepatic and extrahepatic ducts.
– These ducts are typically normal in appearance in patients with PBC.
– In primary sclerosing cholangitis patients, these ducts have a beaded appearance (multiple narrowings along the ducts with areas of widening in between).
– MRCP is noninvasive and safe.
– ERCP is more invasive and carries a 5%-6% chance of causing an attack of acute pancreatitis.
– However, ERCP has the advantage of obtaining cell samples (a process called brush cytology) from the bile ducts.
– Brush cytology is not very accurate.
– It can help to diagnose cholangiocarcinoma.
– During ERCP, doctor can also insert balloons and stents across major areas of narrowing (dominant strictures).
– This is done to relieve obstruction and treat infection.

– Patients with primary sclerosing cholangitis have a high likelihood of also having ulcerative colitis.
– Patients with primary sclerosing cholangitis and ulcerative colitis have higher risks of developing colon cancer.
– Colonoscopy is important to both diagnose ulcerative colitis and for early detection of cancer.
– It is also used to study the precancerous conditions.

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