Primary Sclerosing Cholangitis – Symptoms and Causes
Overview
Primary Sclerosing Cholangitis (PSC) is a serious liver condition that affects the bile ducts. These ducts normally transport bile from the liver to the small intestine to aid digestion.
When someone has PSC, inflammation causes scarring inside these ducts. This makes them hard and narrow over time.
The scarring process gradually leads to significant liver damage. Most people with PSC also have inflammatory bowel disease, particularly ulcerative colitis or Crohn’s disease. This connection suggests possible shared immune system issues.
PSC typically progresses slowly in most patients. Without treatment, it can eventually cause liver failure, repeated infections, and tumors in the bile ducts or liver.
A liver transplant remains the only known cure for advanced PSC, though the disease may return in the new liver for some patients.
Signs and Symptoms
Primary sclerosing cholangitis often gets found before symptoms show up. This happens when routine blood work or X-rays for other reasons reveal liver problems.
Early symptoms that may appear include:
- Feeling very tired
- Itchy skin
- Yellowing of eyes and skin (jaundice)
- Pain in the belly area
Many people who get diagnosed early may feel fine for years. However, no one can predict exactly how quickly or slowly the disease will progress for each person.
As the disease gets worse, you might notice:
- High temperature
- Shivering
- Excessive sweating at night
- Liver enlargement
- Enlarged spleen
- Loss of weight
When To Call a Doctor
See your doctor if you have intense itching that covers large parts of your body and doesn’t stop no matter how much you scratch. You should also make an appointment if you feel extremely tired all the time.
This is especially important if you have ulcerative colitis or Crohn’s disease, which are inflammatory bowel conditions. Most people with primary sclerosing cholangitis also have one of these diseases.
Causes
Scientists still don’t know exactly what causes primary sclerosing cholangitis. Many experts believe the condition might start when the immune system overreacts to an infection or toxic substance in people who have certain genetic factors.
A strong connection exists between primary sclerosing cholangitis and inflammatory bowel disease. This group of conditions includes ulcerative colitis and Crohn’s disease. Many patients have both conditions, though they don’t always appear at the same time.
In some cases, primary sclerosing cholangitis develops years before inflammatory bowel disease shows up. Because of this relationship, doctors typically recommend checking for inflammatory bowel disease when diagnosing primary sclerosing cholangitis.
Risk Factors
Several factors may raise your chance of developing primary sclerosing cholangitis:
Age: While this condition can happen at any age, doctors most often diagnose it in people between 30 and 40 years old.
Gender: Men face a higher risk of developing this disease than women.
Inflammatory Bowel Disease: Many people with primary sclerosing cholangitis also have inflammatory bowel disease.
Heritage: People with Northern European ancestry have increased risk for this condition.
Health Problems
PSC can cause several health issues as the disease gets worse. These problems can be serious and need careful watching by a doctor.
Liver Damage and Failure
When bile ducts become inflamed for a long time, scars form in the liver. This scarring can eventually lead to liver cells dying and the liver stopping its normal work.
Frequent Infections
Scarred bile ducts can slow or block bile flow, causing repeated infections in these ducts. The risk of getting an infection is higher after surgery to widen a scarred duct or remove a blockage.
High Blood Pressure in the Portal Vein
The portal vein carries blood from your digestive organs to your liver. When pressure in this vein gets too high, fluid may leak into your belly (ascites).
Blood may also flow into other veins, making them swell (varices). These swollen veins can bleed easily, which can be dangerous.
Bone Loss
People with PSC may develop weak bones (osteoporosis). Doctors often recommend bone density tests every few years. They also suggest taking calcium and vitamin D supplements to keep bones strong.
Cancer Risks
PSC increases the chance of developing bile duct or gallbladder cancer and colon cancer. People with PSC may also develop colon cancer, especially if they also have inflammatory bowel disease.
If you have PSC, your doctor might test for inflammatory bowel disease even without symptoms. Having both conditions raises your colon cancer risk significantly.
