What is primary sclerosing cholangitis?
"Sclerosing" means scarring and hardening. "-Itis" means inflammation or swelling. "Cholang-" means bile ducts. So cholangitis is inflammation of your bile ducts. Primary sclerosing cholangitis is a disease with swelling, scarring, and narrowing of the bile ducts.
Your bile ducts are tubes that carry bile from your liver to your intestines. Bile is a greenish thick fluid that helps in digestion. Inside your liver are small bile ducts. Outside your liver are larger bile ducts that connect your liver to your intestines.
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If you have primary sclerosing cholangitis, your bile ducts become blocked and eventually your liver stops working
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Primary sclerosing cholangitis is most common in people who have inflammatory bowel disease, particularly men who are in their 40s
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The first symptoms are weakness, tiredness, and itchy skin
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Blocked bile ducts can cause bile duct cancer and cirrhosis
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Doctors treat primary sclerosing cholangitis with medicine for itching and sometimes liver transplant
What causes primary sclerosing cholangitis?
Doctors aren't sure exactly what causes primary sclerosing cholangitis, but it's likely an autoimmune disease (a disease that causes your body’s immune system to attack its own tissues).
You're more likely to get it if you have:
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Inflammatory bowel disease, especially ulcerative colitis
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People in your family with the disease
What are the symptoms of primary sclerosing cholangitis?
Symptoms of primary sclerosing cholangitis include:
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Feeling weak and tired
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Yellow eyes and skin (jaundice)
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Sometimes, pain in your upper belly
Later you may have:
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Osteoporosis (weak bones)
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Bruising and bleeding easily
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Greasy and bad-smelling stool
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Liver problems, such as cirrhosis
Liver failure usually happens about 12 years after diagnosis.
