Primary sclerosing cholangitis

Overview

Primary sclerosing cholangitis is an illness that affects the bile ducts. The digestive fluid bile is transported from your liver to your small intestine by bile ducts. In primary sclerosing cholangitis, inflammation in the bile ducts results in scarring. These scars harden and constrict the ducts, progressively harming the liver. Most persons who have primary sclerosing cholangitis also have an inflammatory bowel condition like Crohn’s disease or ulcerative colitis.

The majority of patients with primary sclerosing cholangitis has slow disease progression. It may ultimately result in liver failure, recurrent infections, and liver or bile duct cancers. The only known treatment for advanced primary sclerosing cholangitis is liver transplantation. In some cases the illness may return in the transplanted liver.

Monitoring liver function, treating symptoms, and, if necessary, performing treatments that temporarily unblock blocked bile ducts are the main components of primary sclerosing cholangitis care.

Symptoms

When a simple blood test or an X-ray conducted for an unrelated ailment reveals liver abnormalities, primary sclerosing cholangitis is frequently identified before symptoms manifest.

Early warning indicators frequently include:

• Jaundice (yellowish discoloration of the skin and eyes)
• Pain in the abdomen
• Itchiness
• Feeling extremely exhausted

For some people who are diagnosed with primary sclerosing cholangitis symptoms don’t appear for years, but there is no way to predict the progression of the disease.

As the disease progresses the following signs and symptoms may appear:

• Losing weight
• Fever and chills
• Enlargement of spleen or liver
• Sweating during the night

If you have severe, unexplained itching on a large portion of your body, itching that does not go away no matter how hard you scratch, schedule an appointment with your doctor. If you consistently feel exhausted no matter what you do, you should also visit your doctor.

If you have inflammatory bowel disease such as Crohn’s disease or ulcerative colitis, it’s crucial to let your doctor know if you experience inexplicable exhaustion and itching.

Causes

The cause of primary sclerosing cholangitis is unknown. In persons who are genetically susceptible to the condition, an immunological response to an infection or toxin may cause the disease.

Most persons who have primary sclerosing cholangitis also have inflammatory bowel disease, which is a catch-all term for conditions including Crohn’s disease and ulcerative colitis.

However, inflammatory bowel disease and primary sclerosing cholangitis don’t typically manifest simultaneously. Primary sclerosing cholangitis may exist for years prior to the development of inflammatory bowel disease. Inflammatory bowel disease should be suspected if primary sclerosing cholangitis is found because colon cancer risk is increased.

In some cases individuals receiving treatment for inflammatory bowel disease are also found to have primary sclerosing cholangitis. Rarely, those with primary sclerosing cholangitis who have a liver transplant later develop inflammatory bowel disease.

Risk factors

Primary sclerosing cholangitis risk factors include the following:

• Age. Although it can happen at any age, primary sclerosing cholangitis is typically identified between the ages of 30 and 40.
• Sex. Men experience primary sclerosing cholangitis more frequently.
• Inflammatory bowel disease. Inflammatory bowel illness is seen in many primary sclerosing cholangitis patients.
• Location. The chance of developing primary sclerosing cholangitis is higher in people with a Northern European ancestry.