Sjögren’s is an autoimmune disease characterized by inflammation of the salivary, tear and respiratory glands. Autoimmune disease means the body’s immune system attacks its own organs and tissues. Dry eyes, mouth and nose are the most common symptoms.

Sjögren’s is the third most common autoimmune disease. It can develop at any age but is most common in women 30 to 50 years old. While Sjögren’s can develop on its own, it also is associated with other connective tissue diseases such systemic lupus or scleroderma.

Arthritis, rash, gastroenteritis and respiratory symptoms are commonly found among people with connective tissue disorders. Sjögren’s can affect many organs, most commonly the lungs, but also the kidney, thyroid and liver.

Based on published research, the frequency of respiratory involvement is variable. About 16% of Sjögren’s patients have pulmonary complications. About 40% have a chronic cough. Doctors should investigate the cough to identify the cause.

People with Sjögren’s can develop PH, chronic cough, blood clots in the lungs, fluid around the lungs, vasculitis and other lung disorders, including inflammation of the lung tissue known as interstitial lung disease.

PH in Sjögren’s can result from damage to the lung tissue, as a reaction to low oxygen levels, or blood-vessel abnormalities in the lungs.

Diagnosing Sjögren’s-related PH

Common signs of Sjögren’s-related PH include cough, shortness of breath, leg swelling, chest pains and fainting. Symptoms that suddenly arise, rather than develop over a period of time, could indicate pulmonary blood clots. When these symptoms occur, pulmonary hypertension should be at the top of the list of possible causes.

Sjögren’s patients with respiratory symptoms should undergo pulmonary function (breathing) tests, chest CTs and x-rays to evaluate the cause. Those with shortness of breath should be evaluated for interstitial lung disease and PH. Although interstitial lung disease (inflammation of the lung tissue) is more common than pulmonary hypertension, the two diseases can overlap.

If tests show signs of PH, such as an enlarged right ventricle, fluid around the heart or elevated pulmonary artery pressure, the patient will need a right heart catheterization. A right heart catheterization can confirm PH and identify whether the PH is related to inflammation of the lung tissue, constriction of the small blood vessels in the lungs, or another cause. Identifying the cause of the PH will help determine appropriate treatment.

If your doctor suspects PH, consider consulting a specialist experienced in assessing and treating PH to develop your care plan and start your treatment. Build a medical team that includes PH specialists, rheumatologists who specialize in autoimmune disorders, dentists and ophthalmologists who communicate well with you and each other.

Treatment options

Treatment depends on the underlying cause of the PH.

If interstitial lung disease is the primary cause, then the primary treatment would be for the lung disorder and autoimmune disease. Treatment for underlying autoimmune disease includes immunosuppressant medications and steroids such as prednisone, azathioprine or mycophenolate, based on the patient’s individual medical issues. PH caused by interstitial lung disease also might respond to inhaled pulmonary vasodilator therapy.

Oxygen therapy is used to improve overall lung function for those with low-oxygen levels. Diuretics (water pills) can decrease swelling and fluid retention, as well as shortness of breath.

Some people with PH and Sjögren’s benefit from targeted therapies for pulmonary hypertension. These therapies should be prescribed by an expert health care professional such as a specialist at a Pulmonary Hypertension Association-accredited PH Care Center.

Treatment is customized according to patients’ medical issues, preferences and PAH severity. Some might need prescription medication to treat dry mouth and relieve other Sjögren’s-related symptoms.

All people with PH should eat a healthful diet and avoid excessive salt intake. Exercise is important to maintain strength, flexibility and conditioning. Exercise such as pulmonary rehabilitation or individual practice can be supervised in consultation with your care team. Avoid exercising to exhaustion or if you experience severe symptoms of PH.

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Information on PH and Sjögren’s is one of a series of PH& brochures available to download as a PDF or to order as a printed brochure.

PH & Sjögren’s