Primary sclerosing cholangitis or PSC is a chronic liver disease in which there is progressive inflammation and fibrosis of the bile ducts. To understand PSC, we need to have an idea about the bile and bile ducts.
Bile is a substance that the liver synthesizes and the gall bladder stores. Bile helps your digestive system digest lipids and absorb fat-soluble vitamins (A, D, E, and K). Bile ducts carry the bile from the liver and gall bladder to do its functions in the intestine. Bile is helpful in the digestive system, but when it accumulates in the liver, it becomes harmful to the liver cell, which occurs in PSC. In PSC, the inflammation and fibrosis cause progressive and gradual narrowing and block of the bile ducts. Also, in PSC, the bile can’t do its functions in the intestine.
Patients with PSC may be asymptomatic and may show symptoms of liver cirrhosis and damage because the disease develops gradually. In PSC, we have a problem other than the liver and bile ducts damage. About 75% of patients with PSC develop inflammatory bowel disease (IBD). Ulcerative colitis and Crohn’s disease are two forms of IBD, but the 1st occurs more with PSC. This association between PSC and IBD puts the patient at a high risk of many cancers, such as liver cancer, gall bladder cancer, and colorectal cancer.
PSC is a rare disease. It affects about one person per 10,000 people. It is more common among patients with IBD; about 5% of these patients have PSC. It affects men more than women, and the diagnosis usually occurs at 30 to 40 years old.
Doctors don’t know what causes PSC. They think that genetic predisposition, environmental triggers, and immune system dysfunction may play a role. Also, doctors didn’t know why PSC and IBD occur with each other.
Diagnosis of PSC begins when your doctor suspects that you have a liver problem. This suspicion comes from a detailed medical history and careful physical examination. Then, your doctor will order investigations to confirm his suspicion and prove PSC.
Your doctor doesn’t have curative treatment for PSC other than liver transplantation, which is the last option. Thus, you will start a management plan that aims to control the symptoms and prevent complications. There are many treatment options to achieve these aims, and your doctor will choose according to your case.
Now, let’s see PSC in more detail; we will know:
- What does cause PSC, and who is at risk of it?
- If you have PSC, which symptoms will you experience, and what are the possible complications?
- When should you see a doctor, and what will your doctor do to diagnose you?
- What are the available treatment options, and how can your doctor control this disease?
Causes of PSC
Scientists didn’t know what exactly causes PSC. Also, they couldn’t understand its pathogenesis well. Some scientists thought that PSC might be autoimmune, but it doesn’t give a valid response to immunosuppressants. Thus, experts believe that some factors lead to it, such as genetic susceptibility, immune system dysfunction, and environmental triggers (like infections and toxins).
There are some risk factors:
- Positive family history: You are at high risk if you have a family member with PSC.
- Male sex
- Age: It occurs at any age, but its diagnosis often occurs between 30 to 40 years old.
- Having an inflammatory bowel disease, especially ulcerative colitis