Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is a progressive liver disease — it worsens over time. PBC is also considered an autoimmune disease, meaning your immune system attacks healthy liver tissues. This creates inflammation that can lead to several complications, or medical conditions that make PBC difficult to treat.

Below, we discuss six complications that may develop from primary biliary cholangitis, plus some diseases that you could develop along with PBC.

1. Weight Loss

Doctors and researchers believe that PBC is an autoimmune disease. According to Mayo Clinic, specialized immune cells known as T cells begin collecting in your liver and create inflammation. These cells attack the healthy cells inside this organ’s small bile ducts, destroying them — leading to one of the reasons you might lose weight with PBC.

Bile ducts that are damaged from inflammation block the flow of bile from the liver to the digestive system. Bile is a digestive fluid needed to break down fatty acids. Without enough of the acids found in bile, you may not be able to absorb the nutrients you need from food. This can lead to malnutrition and, eventually, weight loss.

In addition, advanced-stage liver disease may cause nausea and a lost appetite. As a result, people with PBC often don’t eat enough and lose weight as their disease progresses. This can be a challenge for those getting a liver transplant because they may need to gain weight to prepare for surgery.

If you’ve noticed recent weight loss, be sure to talk to your doctor. They may refer you to a registered dietitian or another specialist who can help.

2. Liver Cancer

Studies show that people with PBC are at a higher risk of developing a type of liver cancer known as hepatocellular carcinoma. Inflammation and cirrhosis in the liver (discussed below) play a role in this increased risk. A meta-analysis of 17 studies found that people with PBC are almost 19 times as likely to develop hepatocellular carcinoma compared with the general population. The authors also noted that those with PBC are at a higher risk of overall cancer, not just liver cancer.

3. Liver Cirrhosis

Over time, the inflammation related to PBC damages other healthy cells in the liver. Your liver tries to heal itself and replace the damaged tissue with scar tissue. Eventually, the scar tissue interferes with your liver function, leading to a condition known as cirrhosis. If you’re living with PBC, you may experience cirrhosis symptoms including fatigue, weakness, and stomach problems such as nausea and vomiting.

If it progresses, cirrhosis can cause further symptoms such as jaundice (yellowing of the skin and eyes), fluid buildup in the abdomen, and encephalopathy (discussed below). Eventually, cirrhosis can lead to liver failure, which can be life-threatening — the best way to treat it is with a liver transplant.

4. Fluid Buildup From Portal Hypertension

Your portal vein carries blood from your stomach, gallbladder, pancreas, and other abdominal organs to your liver. The blood is full of both toxins and nutrients that need to be processed and filtered out. Once your liver processes the blood, it flows back up to your heart.

Cirrhosis in the later stages of PBC creates scar tissue in the liver that blocks blood flow through your portal vein. The blood backs up like a clogged pipe, leading to a condition known as portal hypertension (high blood pressure in the portal vein).

Portal hypertension can lead to many symptoms, including:

• Ascites (fluid buildup in your abdomen)
• Swelling in your spleen and liver
• Edema (fluid buildup and swelling in your feet, ankles, and legs)
• Swollen veins or varices in your abdomen and esophagus (the tube connecting your mouth and stomach)
• Spider angiomas (red blood vessels just below the skin that look like spider webs)

5. Hepatic Encephalopathy

Your liver acts as a filter to remove toxins by breaking down what you eat and drink. Damage from cirrhosis prevents your liver from working properly, causing toxins to build up in your bloodstream. Eventually, the buildup can affect your brain, leading to a condition known as hepatic encephalopathy. One study of 6,375 people with PBC found that almost 6 percent had hepatic encephalopathy.

Symptoms of hepatic encephalopathy include confusion and cognitive impairment (changes in judgment). You may have trouble coordinating movements, balancing, and concentrating on tasks. Some people also experience personality or mood changes.

If you begin noticing symptoms of hepatic encephalopathy, talk to your doctor. Hepatic encephalopathy can be controlled with specific laxatives or antibiotics to help clear extra toxins out of your body.

6. Vitamin Deficiencies

Without enough bile in your digestive system, your body can’t absorb fats properly. Late-stage PBC can also cause deficiencies in fat-soluble vitamins, including vitamins A, D, E, and K. For example, one study followed 255 people with PBC who were treated with ursodeoxycholic acid (a nontoxic bile acid used as a drug, also known as ursodiol). Researchers found that 25 percent of the participants had a vitamin D deficiency.

A lack of these important nutrients can lead to other complications. For example, people with PBC are more likely to develop osteoporosis — weak and brittle bones — because healthy bones require vitamin D as well as calcium. Low vitamin A levels can lead to night blindness, making it difficult to see in low light. If your body can’t absorb enough vitamin K, you may notice you bruise or bleed more easily than normal. Faulty absorption of fats can also cause diarrhea and weight loss.

Your doctor may prescribe supplements to help boost your vitamin levels and lower your risk of complications.

Conditions Associated With Primary Biliary Cholangitis

You may also have a comorbid condition that occurs alongside your PBC. It’s important to note that comorbidities don’t develop as a result of PBC — you have two different conditions at the same time. You may need additional treatments to manage any comorbid conditions.

Studies show that PBC tends to be associated with comorbid autoimmune diseases. In PBC, your immune system attacks your liver and bile ducts and produces proteins known as autoantibodies, which react to your body’s healthy tissues. Around 95 percent of people with PBC have antimitochondrial antibodies (AMA), a biomarker (measurable substance in the body) that helps diagnose PBC.

Autoantibodies known as antinuclear antibodies (ANA) are present in up to 70 percent of PBC cases, according to Wolters Kluwer UpToDate. ANA are also found in comorbid autoimmune diseases associated with PBC, including:

• Sjögren’s syndrome — Affects 40 percent to 65 percent of people with PBC
• Autoimmune thyroid disease — Affects 10 percent to 15 percent of people with PBC
• Limited cutaneous scleroderma — Affects 5 percent to 15 percent of people with PBC
• Rheumatoid arthritis — Affects 5 percent to 10 percent of people with PBC

PBC is also associated with muscle and joint pain from inflammatory arthritis. Between 40 and 70 percent of people with PBC report experiencing musculoskeletal pain. Doctors and researchers have found that there may be a type of arthritis — known as “arthritis of PBC” — that differs from rheumatoid arthritis. This can include pain and swelling of joints in the arms and legs that comes and goes over time.

Sometimes, symptoms of comorbid conditions occur before you receive a PBC diagnosis. For example, you may experience dry mouth and dry eyes associated with Sjögren’s syndrome before your doctor diagnoses you with PBC. Also, around half of people with scleroderma (skin thickening) will experience associated symptoms before their PBC diagnosis.

How Complications and Comorbidities Affect Primary Biliary Cholangitis

Complications and comorbidities of PBC may affect your treatment plan and outlook with the disease. It’s important to work closely with your doctors, including your:

• Primary care doctor
• Gastroenterologist (digestive system specialist)
• Hepatologist (liver specialist)

Together, they can help you manage your symptoms and come up with a treatment plan to slow down liver damage and other complications of PBC.

Talk With Others Who Understand

On myPBCteam, the social network for people and their loved ones living with primary biliary cholangitis, members come together to ask questions, give advice, and share their stories with others who understand.

Are you living with PBC? Have you experienced any complications related to your condition? Share your thoughts in the comments below, or start a conversation by posting on your Activities page.