Primary Sclerosing Cholangitis (PSC) is a rare liver disease that scars the bile ducts. A bile duct is a network of thin tubes that pass through the liver, pancreas, and gall bladder and end with our small intestine. It carries bile secreted in the liver to the small intestine for digestion. PSC scars our bile duct (inside and outside the liver) and makes it narrow, hard, and eventually blocks it out. It results in bile buildup in the liver, causing severe liver damage, recurring infections, or bile duct/liver tumours.
About 80% of people with Primary Sclerosing Cholangitis also have inflammatory bowel diseases (IBD) such as Crohn’s disease and ulcerative colitis. The only known cure for advanced stages of the disease is a liver transplant surgery. In some cases, PSC may recur in the transplanted liver.
No, medical practitioners are unsure of what causes PSC. As mentioned, an inflammatory bowel disease may be linked to liver disease. But, in most cases, PCS exists long before the occurrence of bowel diseases. A combination of the genetic, environmental, or immune systems may also be the cause.
PSC is more common in men than women and is diagnosed between ages 30 and 40. According to the NCBI, people residing in North America and Northern Europe are at higher risk. And in a family with at least one affected person, the relative risk is more.
A person may have PSC for years without exhibiting any symptoms. The condition is ideally diagnosed when a blood test is taken for an unrelated disease. The early signs of Primary Sclerosing Cholangitis include fatigue, itchy skin, jaundice, and severe abdominal pain.
As the disease progresses, the symptoms may turn severe. They include
- Ascites, where fluid collects in the spaces in your abdomen
- Fever and night chills due to infections in the bile duct
- Gastrointestinal (GI) bleeding
- Unexplained weight loss
- Enlarged liver and spleen
- Liver disease is the primary complication as bile duct inflammation leads to Liver cirrhosis and the death of liver cells.
- Bile cannot flow through a blocked-out bile duct, resulting in frequent bile duct infections. Surgical procedures to expand a blocked bile duct will only increase the chance of infections.
- People with inflammatory bowel disease and PSClose 1% of bone mass every year and pose a 75% more risk of developing osteoporosis.
- Cholangiocarcinoma, hepatocellular carcinoma, gallbladder, and colorectal cancer, are linked to PSC and contribute to 50% of patient deaths.
- Elevated pressure in the portal vein that leads to the liver may cause blood to back up in the oesophagus and stomach, causing varices.
A damaged liver grows abnormal liver enzymes and leads to vitamin deficiency. Your healthcare provider may diagnose you with PSC based on your symptoms, family medical history, and blood test reports. If necessary, your doctor will suggest an imaging test or liver biopsy and confirm the same.
There is no known cure to stop or slow PSC. Your healthcare provider may prescribe medications to ease initial symptoms and perform surgery to open blocked bile ducts. A liver transplant is suggested in the case of advanced stages, to which many people respond well.
Be sure to follow the instructions of your liver transplant surgeon when it comes to medications Consume a balanced diet that supplies essential nutrients to help your liver function properly and give yourself a good quality of life.