Primary Biliary Cholangitis – Symptoms and Causes
Overview
Primary biliary cholangitis is an autoimmune condition that causes inflammation and gradual destruction of the bile ducts. This disease was previously known as primary biliary cirrhosis.
Bile plays several important roles in the body. It aids in digestion, helps absorb certain vitamins and fats, and removes cholesterol, toxins, and old red blood cells.
When bile ducts become inflamed (cholangitis), the ongoing damage can lead to permanent liver scarring, or cirrhosis. In severe cases, this may progress to liver failure.
Women are more commonly affected by this condition than men. As an autoimmune disease, the immune system incorrectly attacks healthy cells in the body. Scientists believe both genetic factors and environmental triggers contribute to developing this condition.
Primary biliary cholangitis typically progresses slowly. While there is currently no cure, medications can help slow liver damage, particularly when treatment begins early.
Signs and Symptoms
Many people with primary biliary cholangitis have no symptoms when first diagnosed. Doctors often find the disease through routine blood tests.
For those without symptoms, the disease may take 5 to 20 years to show signs. People who already have symptoms when diagnosed often face more serious outcomes.
Early symptoms typically include:
- Extreme tiredness (fatigue)
- Itchy skin that can be intense and uncomfortable
As the disease progresses, additional symptoms may develop:
| Common Later Symptoms | Advanced Symptoms |
|---|---|
| Jaundice (yellowing of skin/eyes) | Fluid buildup in abdomen (ascites) |
| Dry eyes and mouth | Fatty skin deposits (xanthomas) |
| Pain in upper right abdomen | Darkened skin (hyperpigmentation) |
| Enlarged spleen | Osteoporosis (weak bones) |
| Bone, joint, or muscle pain | High cholesterol levels |
| Swollen feet and ankles | Greasy stools (steatorrhea) |
| Hypothyroidism (underactive thyroid) | |
| Unintended weight loss |
These symptoms appear as the disease damages the bile ducts in the liver over time. The severity and combination of symptoms vary from person to person.
Causes
The exact cause of primary biliary cholangitis remains unknown. However, most healthcare experts classify it as an autoimmune condition, where the body’s defense system attacks its own cells.
Environmental triggers and genetic factors likely play important roles in starting this autoimmune response.
When this happens, white blood cells called T lymphocytes (T cells) begin gathering in the liver.
These cells usually help fight harmful germs, but in primary biliary cholangitis, they incorrectly target and destroy the healthy cells lining the small bile ducts in the liver.
This harmful process begins in the smallest bile ducts and gradually spreads to other liver areas. As liver cells die, they’re replaced by scar tissue (fibrosis).
Over time, this scarring can progress to cirrhosis, a serious condition where extensive scarring makes it difficult for the liver to function properly.
The progression from inflammation to scarring follows this pattern:
- Initial inflammation in small bile ducts
- Spread to surrounding liver tissue
- Death of liver cells
- Formation of scar tissue
- Possible development of cirrhosis
Risk Factors
Several factors can raise your chance of developing primary biliary cholangitis:
- Gender: Women face a much higher risk than men.
- Age: Most cases appear in people between 30 and 60 years old.
- Family History: Having relatives with this condition increases your risk.
- Ethnic Background: People with Northern European ancestry have higher rates, though it affects all ethnic groups.
Scientists believe this disease comes from a mix of genetic factors and environmental triggers. These environmental factors might include:
- Certain infections, like urinary tract infections
- Long-term cigarette smoking
- Working with toxic chemicals in certain job settings
Health Problems from Primary Biliary Cholangitis
Primary biliary cholangitis can lead to many serious health issues as the disease gets worse. These problems affect many parts of the body.
Liver Scarring (Cirrhosis)
When the liver becomes scarred, it can’t work properly. This scarring marks a late stage of the disease. People with cirrhosis often have a poorer outlook and face higher risks of other problems.
Portal Vein Pressure Problems
Blood flowing from the digestive organs enters the liver through the portal vein. Scar tissue can block this flow, causing pressure to build up. This means blood backs up in the system. Also, toxins aren’t filtered properly from the blood.
Swollen Blood Vessels (Varices)
When blood can’t flow normally through the liver, it may back up into veins in the:
- Stomach
- Esophagus (food pipe)
These swollen veins can burst and cause dangerous bleeding that needs emergency care.
Enlarged Spleen
The spleen may swell with white blood cells and platelets when the body can’t filter blood properly.
Stone Formation
When bile doesn’t flow correctly, it may harden into stones in the:
- Bile ducts
- Gallbladder
These stones often cause pain and infection.
Cancer Risk
Liver scarring raises the risk of liver cancer. Regular cancer screening becomes necessary for people with liver scarring.
Bone Problems
People with this disease have a higher chance of developing weak, brittle bones that break easily.
Vitamin Shortages
Without enough bile, the body struggles to absorb:
- Vitamin A (affects vision)
- Vitamin D (affects bone health)
- Vitamin E (affects nerve function)
- Vitamin K (affects blood clotting)
Low levels can cause problems like night blindness and bleeding issues.
Cholesterol Issues
Up to 80% of people with primary biliary cholangitis have high cholesterol levels.
Brain Function Changes
Advanced disease with cirrhosis can cause:
- Personality changes
- Memory problems
- Trouble concentrating
Other Related Conditions
This liver disease is linked to other health problems affecting the:
- Thyroid gland
- Skin
- Joints
- Eyes and mouth (causing dryness in a condition called Sjögren’s syndrome)
