Primary sclerosing cholangitis – Symptoms and treatment

Primary sclerosing cholangitis: Symptoms and treatment

Primary sclerosing cholangitis is a disease that affects the bile ducts. Bile ducts transport the all-important digestive fluid bile from the liver to the small intestine. In patients with primary sclerosing cholangitis, the bile ducts are scarred because of inflammation. Over time, the ducts are hardened by the scars, they become narrow and eventually this leads to extensive liver damage. People with primary sclerosing cholangitis also develop irritable bowel disease, which includes ulcerative colitis or Crohn’s disease. Pronunciation tip: sclerosing – skluh-ROHS-ing; cholangitis – koh-lan-JIE-tis.   

What is primary sclerosing cholangitis?

Primary sclerosing cholangitis (PSC) is a condition of the bile ducts. Bile ducts perform an important function. They carry the digestive liquid bile from the liver to the small intestine. The PSC condition develops in a patient through a chronic inflammation in the bile ducts (cholangitis). Subsequently, this causes extensive scarring (sclerosis). The scarred tissues cause the bile duct to harden and become narrow. This leads to biliary stricture, restricting the flow of bile through the bile ducts. The free flow of bile is now blocked. This, in turn, leads to the bile returning to the liver where it originally came from. Progressively, the damage to the liver becomes extensive.  

Symptoms of primary sclerosing cholangitis

It is possible that an X-ray or a routine blood test for an unrelated medical condition may point to the patient having PSC. In most cases the diagnosis therefore happens without symptoms showing up. 

However, in most other cases, these are the early symptoms that indicate PSC:

• Abdominal pain.
• Itching.
• Fatigue. 
• Yellow skin and eyes (jaundice).

Patients with a PSC diagnosis has been reported to live symptom-free for a long period of time. Yet, as the disease progresses in some people, these addition symptoms do show up:

• Chills and fever.
• Sweating at night.
• Weight loss.
• Enlarged spleen.
• Enlarged liver.

Cause and risk factors

The exact cause of PSC is not known. Medical practitioners do believe though that, in patients who have a predisposition to PSC, an infection or toxin may cause a reaction in their immune system. This reaction may set off the PSC condition. 

People with PSC usually have irritable bowel disease. This includes ulcerative colitis or Crohn’s disease. Even so, it is not necessary that both PSC and irritable bowel disease show up in a patient together. Some patients live with PSC for a long time before reporting symptoms of irritable bowel disease. Doctors usually test for irritable bowel disease in patients with PSC though. This is to factor in and manage the risk of colon cancer in the patient. 

In a few cases, patients with irritable bowel disease also have PSC. In extremely rare cases, a patient with PSC, who has undergone a liver transplant, may develop irritable bowel disease.

A few risk factors exist for PSC:

• People in the age group of 30 to 40 are most likely to develop PSC. Though PSC can affect people of any age group. 
• People with a northern European lineage are most likely to develop PSC.
• It is mostly men who develop PSC.
• Most people with PSC also have irritable bowel disease.

Diagnosis of PSC

Over half the number of people diagnosed with PSC do not report any symptoms at the time of diagnosis. The diagnosis, in fact, happens often when the patient is being tested for another medical condition. So, in most cases, the PSC diagnosis happens by chance. 

When a doctor notices the possible existence of PSC, they may order a blood test/a liver function test and an imaging test. When the blood test reports the presence of high levels of alkaline phosphatase or certain antibodies, it indicates that the body’s immune system is responding differently in the bile ducts. Also, an increased count of white blood cells points to an infection in the liver. The imaging test performed is the magnetic resonance cholangiopancreatography (MRCP) test. This test helps with a detailed visual examination of the biliary tree – the liver, gallbladder and bile ducts. 

Treatment options

PSC cannot be treated presently. That is, there is no treatment to cure the condition or slow down its progress.

Doctors usually attempt managing the symptoms:

• Medication is prescribed to help relieve the itchy skin condition or pruritus.
• Vitamin deficiencies in the patient are made up by prescribing supplements.
• Antibiotics are used to fight infections.

A patient with PSC will require frequent reviews with their doctor to constantly manage the progress of the disease. The doctor may need to perform a procedure, whenever required, to open up blocked bile ducts. This procedure is called ERCP (endoscopic retrograde cholangiopancreatography). It is an endoscopy that allows the doctor to examine the bile ducts and enlarge them without an open surgery. 

Such interventions do not offer a permanent solution though. Over a couple of decades after PSC is diagnosed, the disease progresses to damage the liver extensively. In some cases, it leads to liver failure. A liver transplant then would be the only way help the patient live. 

Living with PSC

A patient having PSC has to make important lifestyle changes. These are aimed at avoiding additional damage to the liver and to reduce the fatigue that the disease causes. The lifestyle changes include:

• Giving up alcohol completely.
• Avoiding processed foods.
• Eating whole foods.
• Sleeping well.
• Reducing stress levels.
• Exercising routinely and adequately.

Conclusion

You cannot prevent PSC. It is will surface without any notice. It is also not a very commonly prevalent condition. It is mostly diagnosed when testing for an unrelated medical condition. This is because the symptoms of the disease do not show up in most people living with it. As soon as it is diagnosed, the patient will do well to immediately make lifestyle changes to save the liver from being damaged any further. Regular reviews with the doctor are also essential to track the progress of the disease and plan interventions accordingly.  

Frequently Asked Questions

1.What is primary sclerosing cholangitis?

Primary sclerosing cholangitis is a disease that affects the bile ducts. Bile ducts transport the all-important digestive fluid bile from the liver to the small intestine. In patients with primary sclerosing cholangitis, the bile ducts are scarred because of inflammation. Over time, the ducts are hardened by the scars, they become narrow and eventually this leads to extensive liver damage.

2.What are the symptoms of PSC?

The early symptoms that indicate PSC are abdominal pain, itching, fatigue, and yellow skin and eyes (jaundice). As the disease progresses in some people, they may have chills and fever, sweating at night, weight loss, an enlarged spleen and enlarged liver.

3.What causes PSC?

The exact cause of PSC is not known. Medical practitioners do believe though that, in patients who have a predisposition to PSC, an infection or toxin may cause a reaction in their immune system. This reaction may set off the PSC condition. 

4.What are the treatment options?

PSC cannot be treated presently. That is, there is no treatment to cure the condition or slow down its progress. Doctors usually attempt managing the symptoms.

5.How do you live with PSC?

A patient having PSC has to make important lifestyle changes. These are aimed at avoiding additional damage to the liver and to reduce the fatigue that the disease causes. The lifestyle changes include giving up alcohol completely, avoiding processed foods, eating whole foods, sleeping well and reducing stress levels.