Though having another autoimmune disease alongside primary biliary cholangitis (PBC) is common, a large study recently published in Clinical Gastroenterology and Hepatology found that these additional conditions don’t seem to make PBC worse.
Researchers found that compared to those who only had PBC, people with PBC and another autoimmune disease responded similarly to treatment and had similar long-term outcomes.
The study included 4,351 people with PBC who were identified through the Dutch PBC Cohort Study between 1990 and 2023. Overall, 27.3% had at least one extrahepatic autoimmune disease, meaning an autoimmune condition affecting an organ or system outside the liver. Hypothyroidism was the most common, followed by Sjögren’s syndrome.
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“A unique strength of our study is that we included a large nation-wide population of patients with PBC, almost eliminating the possibility of selection bias,” the study’s authors said.
Most of the additional autoimmune diseases were already present when PBC was diagnosed. Among the 957 people who had an extra autoimmune disease at that time, 75.8% had one condition and 24.2% had two or more. However, new autoimmune diseases sometimes developed later: About 11% of people who didn’t have an autoimmune disease when they were diagnosed with PBC developed one during the following 10 years.
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The presence of another autoimmune disease did not appear to change how people responded to ursodeoxycholic acid, the standard first-line treatment for PBC. After one year of treatment, about six in 10 people in both the PBC-only and PBC plus another disease groups met the study’s criteria for a good treatment response. The researchers also found no meaningful difference in the likelihood of developing complications from liver disease.
The long-term results were similarly reassuring. After 10 years, about 83% of people with another autoimmune disease and 85% of those without one were still alive without needing a liver transplant. The researchers found no significant difference in liver disease progression, death or the need for a liver transplant between the groups. The four most common individual autoimmune diseases also were not linked to worse PBC outcomes.
For patients, these results suggest that having another autoimmune disease does not necessarily mean that PBC will become more severe or progress faster. However, the researchers emphasize that symptoms can overlap between PBC and other autoimmune diseases. Paying attention to new or unexplained symptoms and evaluating them appropriately may help ensure that conditions outside the liver are recognized and treated.
