With primary biliary cholangitis (PBC), you may not have any symptoms for a long time. But as the illness advances, you could have a range of health problems, from itchy skin and fatigue to osteoporosis and liver cancer. Eventually, PBC can cause liver failure, and you’ll need a liver transplant to save your life.
Early diagnosis and medication help to slow the disease, and many people never reach the later stages. It likely won’t have too much impact on your day-to-day life. But if you’re diagnosed at a later stage or have a more aggressive form of PBC, you’ll need a liver transplant.
How PBC Could Advance
Although PBC typically gets worse in stages, it’s hard to neatly fit the illness into categories. Symptoms like fatigue and itch can happen earlier or later in the disease and be mild or serious at any stage. Doctors have found, though, that more serious symptoms at an earlier stage are a sign that PBC will get worse more quickly.
PBC Stages
Doctors and researchers have divided PBC into four stages based on how serious the damage is to your liver. Here’s a closer look at what you can generally expect at each stage:
Stage 1: Portal stage
During this first stage, you’ll have some damage to your bile ducts. These tiny canals carry bile, a fluid your liver makes that helps your body digest fat, absorb vitamins, and get rid of cholesterol and toxins. A backup of bile harms liver tissues and leads to liver scarring (cirrhosis).
You’ll also have inflammation around the portal area of your liver, where blood vessels and bile ducts come together to help deliver oxygen and nutrients. The liver gets inflamed because white blood cells called T cells or T lymphocytes gather there. Usually, these cells fight off germs like bacteria and viruses to keep you healthy. But in PBC, they attack the healthy cells lining the small bile ducts in the liver instead.
You probably won’t have any symptoms of PBC during this first stage, which can last for a while. Symptoms may start to appear within 5 to 10 years, and once they begin, the average lifespan with PBC is about 10 years. With a successful liver transplant, your 10-year survival rate is about 65%.
Stage 2: Periportal stage
At this stage, you’ll have more inflammation around the portal areas. Inflammation that comes and goes is a normal part of healing. But when it sticks with you long-term, it spreads, damaging and killing liver cells. When this happens, scar tissue (fibrosis) replaces the cells. It narrows and warps your bile ducts and blocks the flow of bile. Bile backs up into your liver and eventually leads to cirrhosis, which affects how your liver works.
Stage 3: Septal stage
Stage three, called the septal stage, involves fibrosis along the thin walls (septa) within the liver. You’ll also have active inflammation within these walls.
Stage 4: Biliary cirrhosis
This is the advanced stage. Here, nodules form on the liver, and you’ll continue to have inflammation. At this stage, you have a higher chance of other health problems due to PBC, such as:
Thinning and weakening of the bones (osteoporosis)
- Liver cancer
- Higher pressure in your portal vein (portal hypertension)
- Enlarged veins (varices)
- Enlarged spleen (splenomegaly)
- Gallstones and bile duct stones
- Low levels of vitamins A, D, E and K
- Personality changes and problems with memory and concentration (hepatic encephalopathy)
- A higher chance of thyroid, skin, and joint disease
- Problems breaking down and absorbing fats (fat malabsorption)
At this stage, you’re also likely to have liver failure and need a liver transplant. This is where a doctor removes your unhealthy liver and replaces it with a healthy donated liver. With a transplant, you’ll probably live an average life span, although the illness could return even years after surgery.
Show Sources
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SOURCES:
Clinical Liver Disease: “Microscopic anatomy of the liver.”
Laboratory Investigation: “Portal tract fibrogenesis in the liver.”
Cleveland Clinic: “Primary Biliary Cholangitis (PBC).”
Mayo Clinic: “Primary biliary cholangitis.”
American Liver Foundation: “Primary Biliary Cholangitis (PBC).”
Pandit, S., Samant, H. Primary Biliary Cholangitis. StatPearls Publishing, 2024.
PBCers Organization: “PBC Disease Stages.”
