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.
If you have primary sclerosing cholangitis, your bile ducts become blocked and eventually your liver stops working
Primary sclerosing cholangitis is most common in people who have inflammatory bowel disease Overview of Inflammatory Bowel Disease (IBD) In inflammatory bowel diseases, the intestine (bowel) becomes inflamed, often causing recurring abdominal pain and diarrhea. The two primary types of inflammatory bowel disease (IBD) are Crohn... read more , particularly men who are in their 40s
The first symptoms are weakness, tiredness, and itchy skin
Blocked bile ducts can cause bile duct cancer Tumors of the Bile Ducts and Gallbladder Tumors, both noncancerous and cancerous, within the bile ducts or gallbladder are rare. Ultrasonography or MRI/MRCP can usually detect a tumor in the bile ducts or gallbladder. These cancers... read more and cirrhosis Cirrhosis of the Liver Your liver is a football-sized organ on the right side of your belly, just below your ribs. It has many important jobs: To make a liquid (bile) that helps your body digest fat To process nutrients... read more
Doctors treat primary sclerosing cholangitis with medicine for itching and sometimes liver transplant Liver Transplantation Liver transplantation is the surgical removal of a healthy liver or sometimes a part of a liver from a living person and then its transfer into a person whose liver no longer functions. (See... read more
What causes primary sclerosing cholangitis?
Doctors aren't sure exactly what causes primary sclerosing cholangitis, but it's likely an autoimmune disease Autoimmune Diseases "Auto" is a medical term for "self." The immune system is your body's defense system. It helps protect you from illness and infection. The immune system usually attacks invading bacteria, viruses... read more (a disease that causes your body’s immune system Overview of the Immune System The immune system is your body's defense system. It helps protect you from illness and infection. The immune system's job is to attack things that don’t belong in your body, including: Germs... read more to attack its own tissues).
You're more likely to get it if you have:
People in your family with the disease
What are the symptoms of primary sclerosing cholangitis?
Symptoms of primary sclerosing cholangitis include:
Feeling weak and tired
Sometimes, pain in your upper belly
Later you may have:
Bruising and bleeding easily
Greasy and bad-smelling stool
How can doctors tell if I have primary sclerosing cholangitis?
Doctors suspect you have primary sclerosing cholangitis from your symptoms and abnormalities they see on liver blood tests. To tell for sure they'll do:
Ultrasound of your bile ducts
A special kind of MRI called MRCP that focuses on your bile ducts
Special blood tests
How do doctors treat primary sclerosing cholangitis?
There's no cure for primary sclerosing cholangitis.
If you don’t have any symptoms, doctors will do blood tests twice a year to check how well your liver is working.
If you have symptoms, doctors treat primary sclerosing cholangitis using:
Medicine to lessen itching and treat infection
A procedure to open your blocked bile ducts