Primary sclerosing cholangitis
Make an Appointment
Talk to DoctorOverview
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.
Diagnosis
The following tests and techniques are used to identify primary sclerosing cholangitis:
- Liver function test. Your doctor can learn more about your diagnosis through a blood test that evaluates your liver function, including the levels of your liver enzymes. Primary sclerosing cholangitis may reveal an increase in alkaline phosphatase.
- Magnetic Resonance Imaging (MRI). The preferred test for identifying primary sclerosing cholangitis is magnetic resonance cholangiopancreatography, which makes images of your liver and bile ducts using MRI.
- Bile duct X-ray. In addition to, or instead of, an MRI, Endoscopic Retrograde Cholangiopancreatography (ERCP), a form of bile duct X-ray, may be required.
Your doctor will inject dye into the region of your small intestine where your bile ducts empty in order to make your bile ducts visible on an X-ray using a flexible tube that is passed through your neck.
If signs and symptoms continue despite no abnormalities on an MRI, an ERCP is the test of choice. If a metal implant in your body prevents you from having an MRI, an ERCP is frequently the first test you undergo.
- Liver biopsy. A portion of liver tissue is removed during a liver biopsy surgery for testing in the laboratory. To obtain a tissue sample from your liver, your doctor will insert a needle through your skin and into the organ.
The degree of liver damage can be determined with a liver biopsy. Only when primary sclerosing cholangitis cannot be definitively diagnosed based on less invasive testing is the test used.
Treatment
Primary sclerosing cholangitis treatments prioritize preventing complications and tracking liver damage. Numerous drugs have been investigated in patients with primary sclerosing cholangitis, but none have been shown to prevent or reverse the liver damage brought on by this condition.
Treatment for itching
- Bile acid sequestrants. The initial line of treatment for itching in primary sclerosing cholangitis involves drugs that bind to bile acids, the compounds thought to be responsible for itching in liver disease.
- Antibiotics. An antibiotic medication called rifampin may be prescribed by your doctor if you have difficulties tolerating a bile acid-binding medication or if it doesn’t work. Rifampin may inhibit the brain’s reaction to molecules in your circulation that cause itching.
- Antihistamines. Primary sclerosing cholangitis-related minor itching may be lessened with the aid of this kind of medication.
Dry eyes and dry mouth are symptoms of liver disease that antihistamines may make worse. Antihistamines, on the other hand, can aid in sleep if itching keeps you awake.
- Opioid antagonists. Medications that act as opioid antagonists, such as naltrexone, may also reduce the itching brought on by liver disease. Similar to rifampin, these medications appear to lessen the itch via working on your brain.
- Ursodeoxycholic acid (UDCA). UDCA, also known as ursodiol, is a naturally occurring bile acid that may lessen liver disease-related itching by improving bile’s absorbability.
Treatment for infections
Bacterial infections are frequently brought on by bile that builds backs up in constricted or blocked ducts. People with primary sclerosing cholangitis may take numerous courses of antibiotics or continue taking antibiotics for extended periods of time to prevent and treat these infections.
You must also take antibiotics before to any procedure that can result in an infection, such as an endoscopic procedure or abdominal surgery.
Nutrition support
Your body has trouble absorbing several vitamins if you have primary sclerosing cholangitis. Despite eating a healthy diet, you may not acquire all the nutrients you require.
Your doctor might advise vitamin supplements that you consume orally or get infused into a vein in your arm. You can take calcium and vitamin D supplements if the condition damages your bones.
Treatment for bile duct blockages
Your bile ducts may get blocked as the condition progresses, but they can also be an indication of bile duct cancer. ERCP can assist in determining the reason. Bile duct blockage can be treated with:
- Balloon dilation. The larger bile ducts outside the liver may get blocked, but this surgery can clear them. Your doctor uses an endoscope to insert a thin tube with an inflatable balloon at its tip (a balloon catheter) into a clogged bile duct. The balloon is inflated after the balloon catheter has been positioned.
- Stent placement. In this operation, your doctor places a tiny plastic tube known as a stent in a blocked bile duct to keep the duct open using an endoscope and connected equipment.
Liver transplant
The only known cure for primary sclerosing cholangitis is a liver transplant. A healthy liver from a donor is substituted for your damaged liver during a liver transplant.
Only those with liver failure or other serious consequences of primary sclerosing cholangitis are candidates for a liver transplant. After a liver transplant, it is rare but possible for primary sclerosing cholangitis to return.
