Primary biliary cholangitis (PBC) is an autoimmune condition that affects the liver’s bile ducts, causing symptoms like itching, dry skin, dry eyes, and dry mouth. Sjögren’s disease is another autoimmune condition that affects glands in the body, with dry eyes and mouth as two of the most common symptoms.
These two conditions can cause similar symptoms and may even occur together, but how each is diagnosed and treated can be very different. In this article, we compare PBC and Sjögren’s disease, why these conditions may occur together, how providers diagnose them, and what care looks like.
People with PBC or Sjögren’s may have overlapping risk factors and symptoms, and these conditions can sometimes occur together. This may be because both conditions have a similar disease process. PBC and Sjögren’s disease are both autoimmune conditions, meaning the immune system mistakenly attacks the body’s own cells, causing inflammation and damage.
In the United States, about a third of people with autoimmune conditions are diagnosed with more than one autoimmune disease.
What’s more, research suggests there’s a relationship between PBC and Sjögren’s disease. One study estimates that about 35 percent of people with PBC also have Sjögren’s. This could affect how the two conditions are evaluated and managed.
Many autoimmune diseases have some genetic and environmental factors that affect whether a person develops each disease. Though the causes of PBC and Sjögren’s are still being studied, there are some overlaps in the risk factors that can lead to these autoimmune conditions.
These include the following factors:
Many people with PBC may not feel any symptoms, but those who do may have some overlap with symptoms of Sjögren’s disease.
One member shared, “Sjögren’s? I’ve had dry mouth for a while. Even when I’m drinking, my mouth is dry. I find this very weird. Yesterday, my eyes got so dry.”
Some symptoms can occur with either condition, while others are more closely linked to PBC or Sjögren’s disease. Symptoms may include:
“Fatigue is the most common early complaint in both, which is why these conditions often get written off for years,” Dr. Harsha Moole, MD, internal medicine physician in Topeka, Kansas, shared with myPBCteam. “Dry eyes and dry mouth are the classic Sjögren’s signs, but PBC can carry its own dryness too, so the two overlap more than people expect,” he said.
For some people, symptoms can get very intense, requiring medical attention. “The itching, burning, and stinging can get out of control. I’ve tried over-the-counter drops, and nothing seems to help,” shared one myPBCteam member about their dry eye symptoms.
Contact your healthcare provider for further evaluation if you have severe joint pain or swelling, intense itching, or dryness in the mouth, eyes, or skin causing pain. Sometimes these symptoms may point to a condition outside of PBC, such as Sjögren’s. Comprehensive testing and evaluation can help your health team find the most appropriate treatments for you.
PBC and Sjögren’s disease can commonly occur together, but they may be diagnosed differently.
According to the American Association for the Study of Liver Diseases (AASLD), PBC is diagnosed when someone has two of these three test results:
Antibody tests are used to diagnose many types of autoimmune diseases, but not all people with PBC may be positive for AMA antibodies. In people who test negative for AMA, doctors may also look for other antibodies linked to PBC. Similarly, antibody tests are used to diagnose Sjögren’s disease, but they may not always be present.
Sjögren’s is diagnosed if people meet certain criteria in their medical history, blood tests, or biopsy tests. Healthcare providers may test for the following blood markers in people with Sjögren’s:
Doctors may also use tests that measure tear and saliva production. These can include the Schirmer test for tear production, eye-surface staining, and a test that measures how much saliva a person makes. A minor salivary gland biopsy may also help nail down the diagnosis.
“Sjögren’s attacks the moisture-producing glands, with positive anti-SSA or SSB antibodies and abnormal tear and saliva tests,” said Dr. Moole. “The liver is usually quiet in Sjögren’s, and the glands are usually spared in PBC, until they are not.”
However, someone can have both conditions, Dr. Moole said, “The two conditions travel together more often than chance would predict, so when a patient has one, it is worth checking for the other.”
How healthcare providers manage PBC and Sjögren’s has some overlap. However, there are some differences to keep in mind when managing them together.
“Autoimmune care is individualized by organ, severity, and what the patient can tolerate,” said Dr. Moole. Your treatment options can depend on your symptoms. This is called symptomatic or supportive treatment.
For example, people with dry eyes may be advised to use artificial tears or prescription eye drops. For dry mouth, drinking water or chewing sugar-free gum may help. Prescription medications can also help the body make more saliva.
Your doctor’s evaluation will determine how to manage Sjögren’s and PBC. The first-line treatment for PBC is usually ursodeoxycholic acid (UDCA, also called Ursodiol). It’s recommended when liver function tests are abnormal. Other medications are recommended when UDCA doesn’t work well or isn’t tolerated.
On top of supportive treatment, people with Sjögren’s may be recommended medications that affect the immune system. Depending on a person’s symptoms and which parts of the body are affected, doctors may use medications such as hydroxychloroquine, corticosteroids, or methotrexate to help control inflammation and immune-system activity.
Keep in touch with your care team if you’re managing both PBC and Sjögren’s together. “When the picture is mixed, a rheumatologist and a hepatologist often work together to sort it out. Sometimes it takes both specialties,” advised Dr. Moole.
Some medications may interact with each other, and others may have specific dosage instructions. For example, if someone takes an aluminum-based antacid for their Sjögren’s-related acid reflux symptoms, it can block the absorption of Ursodiol needed for PBC.
Always share your full medication list, including your over-the-counter and prescription medications, with your healthcare team.
Keep an open discussion with your healthcare providers about your PBC symptoms and any other health concerns. Here are a few questions to ask your provider about PBC and Sjögren’s disease:
These questions can help you start the conversation to better understand your conditions. Your care team can be the best resource to help turn your test results into a plan based on your health history.
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