Primary biliary cholangitis: Symptoms, treatment and more

Primary biliary cholangitis (PBC) has a very fancy name for something pretty simple: your immune
system gets confused and starts attacking the tiny bile ducts inside your liver. Over time, that
damage can lead to scarring and, if untreated, serious liver problems. But here’s the good news:
PBC is usually slow-moving, there are proven treatments, and many people live full, active lives
with it for decades.

In this guide, we’ll break down what primary biliary cholangitis is, the most common symptoms,
how it’s diagnosed, and what treatment and day-to-day life can look like. Think of it as a
conversation with a friend who happens to be obsessed with liver health (minus the medical
degree, of course). For personal decisions, though, your real-life health care team always gets
the final say.

What is primary biliary cholangitis?

Primary biliary cholangitis is a chronic autoimmune liver disease. “Chronic” means it develops
and progresses over years. “Autoimmune” means the body’s immune system mistakenly attacks its
own cellsin this case, the small bile ducts inside the liver.

These small ducts normally carry bilea fluid that helps digest fat and absorb fat-soluble
vitamins (A, D, E, and K)from the liver to the small intestine. When the ducts are injured or
destroyed, bile can’t flow properly. Instead, it backs up in the liver, causing inflammation and
gradual scarring (fibrosis). Over many years, this can lead to cirrhosis and, in some cases,
liver failure.

Who does PBC affect?

PBC can occur in anyone, but it’s most common in:

• People assigned female at birth (about 75–90% of cases)
• Adults between about 40 and 70 years old
• People with other autoimmune conditions, such as thyroid disease, Sjögren’s syndrome, or rheumatoid arthritis

PBC used to be called primary biliary cirrhosis. The name was changed because
many people are diagnosed earlylong before cirrhosis developsand the old name sounded scarier
than necessary.

What causes PBC?

Doctors don’t know the exact cause, but research suggests a mix of:

• Genetic susceptibility: PBC tends to cluster in families.
• Environmental triggers: Things like infections or certain environmental exposures may “flip the switch” in genetically predisposed people.
• Immune system misfires: A hallmark blood test in PBC is the presence of antimitochondrial antibodies (AMA), which target components inside cells and signal that the immune system is attacking bile duct cells.

None of this means you did something wrong. PBC is not caused by drinking alcohol too much,
“eating badly,” or having a certain personality. It’s an autoimmune process, not a lifestyle
failure.

Common symptoms of primary biliary cholangitis

One of the trickiest things about PBC is that it can be completely silent at first. Many people
are diagnosed because of abnormal liver tests done for something elsesay, an annual checkup or
pre-surgery labs.

Early symptoms

When symptoms do appear, the most common early ones include:

• Fatigue: A bone-deep tiredness that sleep doesn’t fully fix.
• Itching (pruritus): Often worse at night, sometimes without a rash. It can be generalized or more intense on the palms and soles.
• Dry eyes and dry mouth: Often linked to overlapping autoimmune conditions like Sjögren’s syndrome.
• Mild discomfort in the upper right abdomen: Where the liver sits.

Later-stage or more advanced symptoms

As PBC advances and liver damage progresses, people may notice:

• Jaundice: Yellowing of the skin and eyes from elevated bilirubin.
• Dark urine and pale stools: Signs of bile not flowing properly.
• Swelling of legs, ankles, or abdomen (ascites): From fluid accumulation.
• Easy bruising or bleeding: From impaired liver function and clotting factor changes.
• Skin changes: Small yellowish cholesterol deposits around the eyes (xanthelasmas) or on the skin (xanthomas).
• Bone problems: Osteopenia or osteoporosis, making fractures more likely.

Not everyone will experience all of these, and symptom severity doesn’t always match how advanced
the disease is. Someone may feel fine but have significant changes on blood tests or imaging, and
vice versa.

How is PBC diagnosed?

There’s no single “PBC scan,” but doctors usually confirm the diagnosis using a combination of:

1. Blood tests

• Liver enzymes: Alkaline phosphatase (ALP) is often significantly elevated. Other enzymes like AST and ALT may also be elevated.
• Antimitochondrial antibodies (AMA): Present in about 90–95% of people with PBC and highly suggestive of the diagnosis.
• Other antibodies: Such as ANA or anti-gp210 and anti-sp100 in some cases.

2. Imaging

An ultrasound or other imaging study helps rule out other causes of cholestasis, such as large
bile duct obstruction (gallstones, tumors) or other structural liver diseases.

3. Liver biopsy (sometimes)

A liver biopsy isn’t always required, but your doctor may recommend it if:

• Blood work is unclear or atypical
• Other liver diseases (like autoimmune hepatitis or nonalcoholic fatty liver disease) are suspected
• Staging the amount of fibrosis would change management

Newer non-invasive methods, like transient elastography (FibroScan), can help estimate liver
stiffness and scarring, sometimes reducing the need for biopsy.

Treatment options for primary biliary cholangitis

There’s currently no cure for PBC, but there are effective ways to slow disease
progression, protect your liver, and manage symptoms. Treatment plans are highly individualized,
so what you read here is a frameworknot a substitute for medical advice from your own clinician.

First-line medication: ursodeoxycholic acid (UDCA)

The standard first-line treatment for PBC is ursodeoxycholic acid (UDCA), also
called ursodiol. It’s a bile acid that:

• Improves bile flow out of the liver
• Reduces liver enzyme levels
• Delays or reduces progression to cirrhosis
• Improves survival and can delay the need for liver transplantation

UDCA is usually taken lifelong, at a dose based on body weight. Many people tolerate it well, but
regular follow-up blood tests are essential to check how well it’s working.

Second-line and add-on therapies

If liver tests do not improve enough after being on UDCA for a while, doctors may consider:

• Obeticholic acid (OCA): A second-line medication that can be added to UDCA or
  used alone if UDCA isn’t tolerated. It works by activating a receptor that regulates bile
  production and flow. It’s not suitable for everyoneespecially those with advanced cirrhosis
  so careful monitoring is needed.
• Fibrates (such as fenofibrate or bezafibrate): Originally cholesterol-lowering
  drugs, they’re increasingly used off-label in PBC for people who respond poorly to UDCA, as
  they may improve cholestatic markers in some patients.

Your hepatologist will typically follow guideline-based algorithms (such as those from the
American Association for the Study of Liver Diseases) to decide when to add or adjust
medications.

Managing symptoms and complications

Treating PBC is not just about numbers on a lab reportit’s also about how you feel day to day.
Common symptom-focused strategies include:

• Itching (pruritus): First-line options often include bile acid–binding resins
  such as cholestyramine. Other medications like rifampin, opioid antagonists (e.g.,
  naltrexone), or certain antidepressants may be used if itching is severe.
• Fatigue: Unfortunately, there’s no magic pill for PBC-related fatigue. Doctors
  often focus on ruling out and treating other contributors like anemia, thyroid disease, sleep
  apnea, or depression.
• Bone health: Because PBC raises the risk of osteoporosis, many people are
  advised to get adequate calcium and vitamin D, undergo bone density testing, and sometimes take
  bone-protective medications.
• Cholesterol and fat-soluble vitamins: PBC can cause abnormal cholesterol
  levels and interfere with absorption of vitamins A, D, E, and K. Your care team may monitor
  cholesterol and vitamin levels and recommend supplements if needed.

Liver transplantation

For a minority of people, PBC eventually progresses to end-stage liver disease despite optimized
medical therapy. At that point, liver transplantation may be recommended. It can
dramatically improve symptoms and survival, and outcomes for PBC after transplant are generally
excellent.

Transplant evaluation is usually considered when:

• There is significant cirrhosis with complications (ascites, variceal bleeding, encephalopathy)
• Jaundice and lab abnormalities are severe and persistent
• Quality of life is severely impaired by symptoms despite treatment

Living well with primary biliary cholangitis

PBC is serious, but it doesn’t have to define your life. Many people work, travel, raise
families, and pursue hobbies for decades after diagnosis. Here are practical strategies often
recommended by experts and patient organizations.

Daily lifestyle choices that support your liver

• Avoid alcohol: Because your liver is already working harder than it should,
  alcohol adds unnecessary stress.
• Discuss medications and supplements: Some drugs and herbal products are
  potentially harmful to the liver. Always check with your doctor or pharmacist before starting
  anything new.
• Healthy eating pattern: Focus on a balanced diet rich in fruits, vegetables,
  lean proteins, whole grains, and healthy fats. There’s no single “PBC diet,” but a heart- and
  liver-friendly approach is usually advised.
• Stay active at your own pace: Regular physical activity can help with energy,
  mood, bone health, and weight maintenance. Even short walks count.
• Protect your bones: Weight-bearing exercises, not smoking, and making sure
  you’re getting enough vitamin D and calcium all help protect bone density.

Emotional and social health

Chronic illness can feel lonely, even if your labs are “stable.” It’s completely reasonable to
feel anxious, frustrated, or worried about the future.

• Support groups: Many people find online or in-person PBC communities helpful
  for swapping tips and feeling understood.
• Mental health support: Talking with a therapist, counselor, or social worker
  familiar with chronic illness can be a game changer.
• Open communication: Let family and close friends know what PBC is and how it
  affects youespecially the invisible symptoms like fatigue and itching.

Smart questions to ask your health care team

Bringing a short list of questions to appointments can help you feel more in control. Examples:

• What stage is my PBC, and how are you measuring that?
• How well am I responding to UDCA or other treatments?
• How often should I have blood tests and imaging?
• What can we do about my fatigue or itching?
• Do I need screening for complications like osteoporosis or varices?
• Are there any clinical trials or newer therapies I should know about?

Remember, this article is for general education. Your own situation may be more complex, so always
check details and decisions with your liver specialist or primary care clinician.

Real-world experiences: living with PBC day to day

Statistics and guidelines are helpful, but life with primary biliary cholangitis happens in the
messy, in-between spaces: school drop-offs, Zoom meetings, grocery runs, and 3 a.m. itch attacks.
While everyone’s journey is different, many people with PBC describe common phases and coping
strategies.

“You have what?” the diagnosis shock

For many people, the story starts with routine blood work. Maybe a primary care doctor notices
an elevated alkaline phosphatase on a yearly physical and orders more tests. The person feels
fine, so the idea of “liver disease” lands like a plot twist nobody asked for.

It’s completely normal to cycle through disbelief, fear, and angerespecially when you’re told
PBC is chronic and autoimmune, and that treatment is lifelong. Many patients say that getting a
clear explanation of what PBC is (and what it isn’t) from a hepatologist or liver nurse
specialist helps shift things from “mystery threat” to “challenging but manageable condition.”

Learning the new normal

Once treatment startsoften with UDCAdaily life becomes a mix of pill schedules and regular lab
checks. Some people barely notice a difference; others feel more tired or emotionally drained as
they process what the diagnosis means for work, family, and long-term plans.

A common theme is trial and error. One person might discover that taking UDCA with food helps
their stomach. Another might figure out that scheduling blood tests on the same day each month
makes it easier to track trends. People often talk about choosing a small notebook or phone app
to record lab results, medication changes, and questions for upcoming visits. Over time, that
record can make conversations with the care team more focused and productive.

Managing the invisible symptoms

Itching and fatigue can be especially hard to explain to others. An employer or family member
may look at you and say, “But you don’t look sick.” Meanwhile, you’re counting down the minutes
until you can lie down, or trying not to scratch your skin raw during a meeting.

People with PBC often share small, practical tricks:

• Using fragrance-free moisturizers and lukewarm showers to avoid drying the skin
• Wearing soft, breathable fabrics to reduce irritation when itch is intense
• Planning demanding tasks for times of day when energy is usually higher
• Building “rest breaks” into the day without feeling guilty

For some, it helps to come up with a simple explanationsomething like, “I have a chronic liver
condition that makes me very tired sometimes. I’m okay, but I need to pace myself.” That can
open the door to support instead of skepticism.

Finding support and hope

Another recurring theme is the power of connection. People often say that the first time they
meet or message someone else with PBC, they feel an immediate “Oh, you get it.” Online support
communities, patient organizations, and educational webinars can offer:

• Real-world tips for navigating side effects and lifestyle changes
• Ideas for questions to ask doctors
• Reassurance from people who have lived with PBC for 10, 20, or 30+ years

Many patients also talk about reframing their outlook. Instead of seeing PBC as a countdown
clock, they see it as a reason to prioritize what matters: spending time with loved ones,
protecting their energy, and taking better care of their health. Some describe the diagnosis as
an unwelcome wake-up call that led to healthier routinesmore sleep, better food, less smoking,
and a more honest relationship with stress.

Working with your care team as a partner

Over time, people living with PBC often shift from feeling like passive “patients” to being
active partners in their care. They learn what their key lab numbers mean, understand why
specific medications were chosen, and feel more comfortable asking, “What are our options?”

That partnership matters. PBC management is a long game, and your relationship with your care
teambuilt on trust, clear communication, and shared decision-makingcan be just as important as
any single medication. You bring your lived experience; they bring clinical expertise. Together,
you can design a plan that aims not just for longer life, but better life.

Bottom line

Primary biliary cholangitis is a chronic autoimmune liver disease that targets the small bile
ducts, but it’s not an automatic sentence to severe illness. Early diagnosis, guideline-based
treatment with medications like UDCA and, when needed, second-line agents, plus ongoing
monitoring can dramatically change the trajectory of the disease.

If you’ve been diagnosed with PBCor suspect you might have itthe most important next steps are
to work closely with a knowledgeable health care team, ask questions, and build habits that
support both your liver and your overall well-being. Information is power, but partnership is
what turns that power into action.