Systemic lupus erythematosus (SLE or lupus) is a disease that affects a person’s immune system. People with lupus may develop rashes, arthritis, kidney trouble and lung or heart problems. In some very rare cases they may develop Group 1 PH (pulmonary arterial hypertension, or PAH). It is estimated that about 2% of people with lupus will develop PAH, although it is not clear whether lupus causes PAH or whether having lupus makes developing PAH more likely.
The initial screening for all types of PH includes an echocardiogram, which estimates the blood pressure in the lungs and evaluates heart function. More detailed testing, including a right heart catheterization, is required for an accurate diagnosis of PAH. A careful and detailed evaluation is important because lupus can cause other problems, including heart inflammation, lung scarring and blood clots.
Download the Pulmonary Hypertension Association (PHA)’s free patient brochure to learn more about PH risks and treatment options.