Primary Biliary Cholangitis
Primary biliary cholangitis or PBC is a rare, autoimmune liver disease where chronic inflammation of the bile ducts causes them to scar and narrow which makes it harder for bile to flow through. In turn, bile backs up in your liver, damaging the liver tissue and causing it to scar.
Over time, scar tissue gradually replaces the healthy tissue and your liver function declines, a process known as cirrhosis.
Understanding the disease name
Primary: A disease that starts suddenly and without a clear source
Biliary: Related to or affecting the bile ducts in the liver
Cholangitis: Inflammation of the bile ducts
PBC vs PSC
These conditions are very similar with one major difference. PBC affects only your intrahepatic bile ducts (the bile ducts within your liver) whereas PSC, primary sclerosing cholangitis, affects all of your liver bile ducts, including your extrahepatic ducts.
Causes
PBC is believed to be an autoimmune liver disease that is caused by the body’s immune system attacking the bile ducts. The cause for this attack is not known, but researchers believe it is a combination of environmental factors and genetics.
Symptoms
In the early stages, symptoms are rare, but as the disease progresses signs and symptoms begin to appear. You may experience:
Early symptoms
- Fatigue
- Itchy skin
Late symptoms
- Jaundice
- Dry eyes and mouth
- Pain in the upper right abdomen
- Swelling of the spleen
- Bone, muscle or joint pain
- Swollen feet and ankles
- Buildup of fluid in the abdomen due to liver failure
- Darkening of the skin unrelated to sun exposure
- Weak/brittle bones
- High cholesterol
- Diarrhea that may include greasy stools
- Weight loss
- Fatty deposits, called xanthomas, on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees
Diagnosis
In most cases, PBC is diagnosed incidentally during routine liver testing. When it’s clear you have liver damage, your doctor may order additional blood work that will test for PBC. It is typical to have imaging of your liver and bile ducts to determine how advanced the disease is.
Treatment
There is no cure for PBC, but the progression of the disease can be slowed with medication.
These medications work to clear bile from your liver and will improve your overall condition. Individual symptoms such as itching, can be managed with other medications.
If medications don’t work to improve your condition and your liver function continues to decline, your doctor may talk to you about placing you on the liver transplant list.
Liver transplants have shown to have excellent outcomes for patients with PBC.
Routine imagining (2-3 per year) will be necessary for monitoring disease progression.
What happens as PBC worsens?
- Your body beings attacking your bile ducts ⟶ Causing a buildup of toxic bile acid (cholestasis)
- Cholestatsis ⟶ Causes further inflammation an damage to the liver
- Ongoing cell damage leads to harmful scarring (fibrosis) ⟶ Which can result in cirrhosis over time
- Cirrhosis is permanent liver damage ⟶ End stage liver disease is possible, which can lead to transplant
Prognosis
With an earlier diagnosis, many people are able to effectively control their symptoms with medications, except fatigue, which remains difficult to treat. Later stage discoveries of PBC can mean more aggressive symptoms and progression of the disease.
On average, it takes 15 to 20 years for PBC to reach the terminal stage. This is when a liver transplant may become necessary.
How can I take care of myself?
Keeping your liver healthy for as long as possible is key. Your medical team may recommend:
- Quit smoking, drinking and drug use
- Eat whole foods as much as possible
- Limit processed foods and saturated fats
- Take a brisk walk every day
- Incorporate strength training a few times a week