Primary sclerosing cholangitis is a chronic disease that slowly damages the liver. It begins by causing inflammation within the bile ducts, which leads to progressive scarring that blocks the flow of bile. This can result in further damage, such as cirrhosis, liver cancer, and liver failure.
- The causes of primary sclerosing cholangitis are not known. It may be linked to genetics, an immune system reaction, changes in gut bacteria, or some combination of these.
- Most patients do not experience any symptoms when diagnosed. But as damage to the liver becomes more severe, serious health complications can emerge.
- While there is no cure, various treatments are available that can slow the progression of the disease, relieve symptoms, and prevent complications.
The causes of primary sclerosing cholangitis are still not well understood. Researchers currently think it may be linked to a genetic predisposition to the disease, an immune system response to a foreign substance or toxin, changes in the gastrointestinal bacteria (also called gut bacteria or biome), or some combination of these.
Several factors are known to increase the likelihood of developing primary sclerosing cholangitis:
- Age: People between the ages of 30 and 50 are most likely to develop this condition.
- Gender: Men are more likely than women to develop primary sclerosing cholangitis.
- Geography: Primary sclerosing cholangitis is most often found in northern Europe and North America.
- Inflammatory Bowel Disease: Rates of primary sclerosing cholangitis are highest in those with inflammatory bowel disease.
A majority of patients with primary sclerosing cholangitis do not have any symptoms when they are diagnosed. However, as the disease progresses and liver damage becomes more severe, more symptoms will become likely. The length of time this takes will be unique for each patient.
Early symptoms may include the following
Later symptoms may include the following:
- Abdominal pain
- Jaundice (yellowing of eyes and skin)
- Weight loss
Diagnosing primary sclerosing cholangitis usually begins with a physical examination. This will involve checking for any signs of liver inflammation and asking about family medical history and any personal medical history of inflammatory bowel disease and/or autoimmune disease.
The following are some other common ways to diagnose primary sclerosing cholangitis:
- Blood Tests: These test for abnormal levels of liver enzymes, which can be a sign of liver and/or bile duct damage. They may also test for high levels of antibodies and white blood cells, both of which can be a sign of primary sclerosing cholangitis.
- Imaging Tests: These form an image of the liver and bile ducts so that doctors can better detect signs of disease. These tests may include a computerized tomography (CT) scan, an ultrasound, or an MRI (magnetic resonance imaging) test.
- Liver Biopsy: This is a surgical procedure that removes a tiny portion of liver tissue so that doctors can examine it under a microscope to determine what is wrong.
There is no cure for primary sclerosing cholangitis. Instead, the goal of treatment is to alleviate the symptoms and complications of the condition as it grows worse. The following are some of the most common treatments:
- Antibiotics: These are used to treat bile ducts infections, as well as itchy skin.
- Antihistamines: Doctors may prescribe over-the-counter medications to help treat itchy skin.
- Balloon Dilation: This is used to treat blocked bile ducts. In this procedure, surgeons inflate a small balloon inside a blocked bile duct to keep it open.
- Dietary Supplements: Vitamins such as A, D, E, and K may be prescribed to patients whose liver damage makes it hard to absorb adequate nutrients from their food.
- Stent: These are used to treat blocked bile ducts. In this procedure, surgeons place a small plastic tube (a stent) inside a blocked bile duct to keep it open.
- Liver Transplantation: In severe cases, a liver transplantation may be necessary. This involves surgically removing the diseased liver and replacing it with a healthy donor liver. In some cases, primary sclerosing cholangitis can recur in the new liver. Learn more about liver transplantations.
The outlook for those diagnosed with primary sclerosing cholangitis depends on multiple factors, such as the patient’s age, the severity of their liver damage, and the size of their spleen.
Liver transplantation remains the most effective way to increase the long-term survival rate of patients with this condition. However, making healthy lifestyle changes can also help reduce symptoms and complications. These include regularly exercising, following a healthy diet, and reducing alcohol consumption.
Our liver specialists are well-versed in diagnosing primary sclerosing cholangitis and helping patients manage symptoms and complications. We are also a leader in liver transplantations.
Call us at (877) LIVER MD/ (877) 548-3763 or use our online form to schedule an appointment.