Pulmonary hypertension is a chronic and progressive condition that can make physical activity increasingly difficult. Patients with the condition have high pressure in the blood vessels of the lungs or the pulmonary arteries. Left untreated, pulmonary hypertension (PH) can have serious complications, such as heart failure.

The World Health Organization (WHO) has classified pulmonary hypertension into five groups based on the underlying cause of the disease. Group 1, also called pulmonary arterial hypertension (PAH), is caused by the narrowing of the pulmonary arteries, which makes it more difficult for the blood to flow through the lungs.

One of the conditions PAH is associated with is systemic lupus erythematosus, or lupus. Lupus is an autoimmune disorder, a condition where the body’s immune system attacks its own tissues and organs.

How are lupus and PAH related?

Lupus and PAH are two separate conditions, and PAH does not cause or increase the risk of developing lupus.  However, patients with lupus have a low chance of developing PAH. The Pulmonary Hypertension Association estimates that between 0.5 to 9 percent of lupus patients may have PAH.

The exact relationship between lupus and PAH is not well understood, but it is known that lupus can cause a number of complications that could increase the likelihood of, or potentially cause, PAH to develop. This could be through more than one mechanism, including:

Symptoms and diagnosis of lupus-associated PAH

An early diagnosis and treatment of both lupus and PAH can have a significant impact on the prognosis of the disease.

Lupus is a complicated condition to diagnose because the symptoms are similar to those of many other conditions. The main symptoms include joint pain and stiffness, extreme tiredness, skin rashes, weight loss, swollen glands, light sensitivity, and poor circulation.

Once the symptoms have been identified and other conditions have been ruled out, the most common method of diagnosing lupus is through blood tests to check for high levels of certain immune proteins, called antibodies.

Lupus patients are not routinely tested for PAH because the risk of developing lupus-associated PAH is quite low. Therefore, if any of the common symptoms of PAH develop, the patient should discuss this with a medical professional immediately. These include struggling with physical activity, swelling of the feet and ankles, increased shortness of breath and fatigue, chest discomfort or pain, and dizziness or fainting. Other complications of lupus may also cause these symptoms, such as kidney, heart, and lung dysfunction, so additional diagnosis will be required.

Lupus-associated PAH is diagnosed in the same way as pulmonary hypertension once the symptoms have been identified. Multiple tests may be carried out, but a test called right heart catheterization is currently considered the most accurate method of diagnosing lupus-associated PAH. This test measures the pressure in the pulmonary arteries and heart. It is often combined with echocardiography, an imaging test that looks at the heart and lungs, and how well they are functioning.

Other causes of PH should also be investigated to ensure the most appropriate treatment is given. For example, a chest computerized tomography (CT) scan produces images of the lungs, heart and blood vessels and can help identify the presence of blood clots.

Treating lupus-associated PAH

Lupus-associated PAH can often be managed using the same medications prescribed to PAH patients. These often have to be taken along with medicines already prescribed for lupus; a medical professional will be able to help design a treatment plan that best fits individual patients.

There are several therapies that can relieve the symptoms of pulmonary hypertension, including diuretics to reduce swelling and oxygen therapy. Several PAH-approved therapies can widen the pulmonary arteries to reduce pressure and improve oxygen exchange.

Other underlying causes of PAH should be treated separately. For example, anticoagulant medication can reduce and prevent blood clots.

Patients with lupus-associated PAH may also benefit from immunosuppressive therapies in combination with standard PAH therapies. These medications reduce or prevent inflammation caused by an overactive immune system, and may prevent additional damage to the blood vessels in the lungs.

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Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

2 comments

  1. Barbara Bayer says:

    I have Lupus, I was diagnosed about 10 years ago. Two years ago I was diagnosed with PAH. It is tough dealing with both diseases. I am only 49 and I do not know if I will see 55 or older because how PAH is such a deadly and progressive disease. I also have Fibromyalgia and Hypothyroidism.

    Since my Lupus diagnoses my body has slowly gone down hill. One can only pray that there will be cure.

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