Study Shows Pulmonary Arterial Hypertension Complications Affect Sjögren’s Syndrome Patients

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PAH and Sjögren’s SyndromeToday, four million Americans live with a chronic autoimmune disease known as Sjögren’s Syndrome (SS). The condition causes mucous membranes and other glands to function improperly, triggering a series of complications in several organs. According to a recent study, one of these complications is the onset of pulmonary arterial hypertension (PAH) in Sjögren’s syndrome patients, who otherwise present with no condition affecting their immune system.

PAH is characterized by a higher risk for heart failure, pulmonary edema, irregular heartbeat, and sudden death. In response to the significant risks that the onset of pulmonary arterial hypertension adds to SS patients, Senol Kobak from the Department of Rheumatology at Sifa University in Izmir, Turkey, led a study to discover if there is an actual relationship between Sjögren’s Syndrome diagnosis and PAH development.

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The study included 47 patients diagnosed with Primary Sjögren’s Syndrome (without any other condition affecting their immune system) with an average age of 48 and a 5-year duration of the disease. In addition, none of the participants had a previous reported exposure to environmental silica and asbestos, which could interfere with the results of the study.

The participants were given a series of tests, including a chest x-ray, urine tests, and ultrasounds of their chest. These last tests administered were used to measure participants’ lung function and the blood pressure in the pulmonary artery in an effort to diagnose asymptomatic PAH.

The findings revealed that a 23% of the patients had asymptomatic pulmonary arterial hypertension. Interestingly, it was more common in younger female patients, recently diagnosed with SS. Patients with PAH had an average age of 49 years, and had been diagnosed with Sjögren’s Syndrome for 4 years, while patients without PAH  had an average age of 59 years, with a 10-year diagnosis. No factors other than age, gender, and disease duration were associated with the PAH diagnosis.

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Given the results, Dr. Kobak acknowledged that there were limitations in the study that, together with the fact that the data conflicted with previous studies, would suggest that a larger, more expansive study is needed to further corroborate results. Particularly at issue with the study was the fact that PAH is typically diagnosed using an invasive catheterization technique, and not the ultrasound method used in the study. In addition PAH diagnoses in participants were both mild and asymptomatic. Further studies will need to determine if any conclusions can be drawn from Dr. Kobak’s work.

A Conversation With Rare Disease Advocates