When we think about Sjögren’s disease, dry eyes and dry mouth are often the first symptoms that come to mind. However, Sjögren’s is a systemic autoimmune disease, meaning that inflammation associated with the disease can affect other parts of the body, including, in rare cases, the heart.
Heart involvement is uncommon
According to The Sjögren’s Book, involvement of the heart is rare in people with Sjögren’s disease. One of the cardiac problems described is pericarditis, an inflammation of the thin sac, or lining, that surrounds the heart.
Pericarditis can cause a small amount of fluid to collect around the heart. This is called a pericardial effusion. In many people with Sjögren’s, a small effusion may cause no symptoms and may only be discovered incidentally during an echocardiogram (ultrasound of the heart).
When pericarditis does cause symptoms, they may include:
- Chest pain, often on the left side
- Chest pain that changes with body position
- Pain that may be more noticeable when lying down
- Shortness of breath in some cases
- Other symptoms associated with inflammation
Most cases of pericardial involvement are not severe. However, a very large amount of fluid around the heart can, in rare circumstances, interfere with the heart's ability to pump effectively. For this reason, unexplained or persistent chest symptoms should always be discussed with a healthcare professional.
Sjögren’s, lupus and the heart
People who have both Sjögren’s disease and lupus may be more likely to develop symptomatic pericarditis, particularly during a lupus flare. Pericardial effusions can also occur in people with rheumatoid arthritis and Sjögren’s.
Treatment depends on the cause and severity of the inflammation. In the source material, corticosteroids are described as a treatment for small- to medium-sized pericardial effusions, while larger effusions may require drainage of the fluid.
Congenital heart block and Sjögren’s antibodies
Another important connection between Sjögren’s and the heart involves pregnancy. Some people with Sjögren’s have anti-Ro (SSA) and/or anti-La (SSB) antibodies. These antibodies can cross the placenta during pregnancy and, in rare circumstances, affect the developing fetal heart.
This can lead to congenital heart block, a condition in which the electrical signals that regulate the baby's heartbeat are disrupted. The risk is relatively low, but it is an important consideration for pregnant individuals who have anti-Ro or anti-La antibodies.
When a woman with Sjögren’s and anti-Ro antibodies becomes pregnant, the risk of congenital heart block is approximately 1–2%. The risk is higher when there has been a previous pregnancy affected by congenital heart block. Because of this, pregnancies involving anti-Ro and/or anti-La antibodies may require closer monitoring, particularly during the weeks when fetal heart block can develop.
What does this mean for people with Sjögren’s?
Heart involvement is not a common feature of Sjögren’s disease, and most people with Sjögren’s will not develop serious heart complications as a result of the disease. Nevertheless, because Sjögren’s can affect multiple organs, regular medical follow-up is important.
If you experience new, persistent or unexplained chest pain, shortness of breath, a racing or irregular heartbeat, fainting, or other concerning symptoms, speak with your healthcare provider promptly. These symptoms can have many possible causes, and appropriate evaluation is important.
* summary courtesy of The Sjögren's Book, Fifth Edition, Daniel J. Wallace, MD
