Rheumatic Diseases Associated With Pulmonary Arterial Hypertension

Senior man chest pain
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Pulmonary arterial hypertension is a serious condition for which there are treatments but no cure, according to the National Heart, Lung, and Blood Institute. Secondary pulmonary arterial hypertension can be caused by or occurs because of another secondary condition.

Pulmonary Hypertension Causes

Pulmonary arterial hypertension (or high blood pressure in the lungs) is an uncommon but very serious medical condition. This problem, unrelated to increased systemic blood pressure (hypertension), is caused by a loss of lung volume as well as loss of flexibility (elasticity) of the pulmonary artery whose function is to return blood to the lungs for oxygenation.

Rheumatic Diseases Associated With Pulmonary Hypertension

Pulmonary hypertension is associated with a number of conditions. In patients who have extensive scarring of the lungs (fibrosis as seen in scleroderma and sometimes rheumatoid arthritis), there is diminished lung capacity so the heart has to increase the pressure necessary to push the blood through the pulmonary artery to the lungs. Other patients develop increased pulmonary artery pressures due to narrowing of the artery. This can be seen in conditions such as scleroderma (often in the limited form called CREST) or less commonly lupus (systemic lupus erythematosus). Recently this type of pulmonary hypertension was found to be associated with diet drugs that are no longer on the market.


Symptoms of this condition include shortness of breath and chest pain. Diagnosis can be suggested by a non-invasive ultrasound of the heart (echocardiogram). Cardiac catheterization (heart catheterization) which has some medical risks is often done to confirm the diagnosis.


In the past, there was little treatment available for this problem which carried a poor prognosis. Fortunately, there are newer therapies available to help patients with their condition including:

  • epoprostenol (short-acting drug that requires continuous infusion with a large IV)
  • treprostinil (administered by continuous subcutaneous infusion)
  • bosentan (given orally)
  • sildenafil (given orally)
  • iloprost (inhaled)

Additional therapies include oxygen, blood thinners, diuretics, and cardiopulmonary rehabilitation. If diagnosed with this problem, it is best to see a lung doctor who has expertise in this disorder.

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  • Answer provided by Scott J. Zashin, M.D., clinical assistant professor at University of Texas Southwestern Medical School, Division of Rheumatology, in Dallas, Texas. Dr. Zashin is also an attending physician at Presbyterian Hospitals of Dallas and Plano. He is a fellow of the American College of Physicians and the American College of Rheumatology and a member of the American Medical Association. Dr. Zashin is author of Arthritis Without Pain - The Miracle of Anti-TNF Blockers and co-author of Natural Arthritis Treatment.