Systemic lupus erythematosus affects a person’s immune system. People with lupus can develop rashes, arthritis, kidney trouble and lung or heart problems. About 2 percent of people with lupus develop pulmonary arterial hypertension.
Diagnosing Lupus-associated PAH
The initial screening for lupus is a blood test called an antinuclear antibody. Many people who don’t have lupus will have positive ANA results. If the test is positive, a focused physical examination, more blood tests and a visit with a rheumatologist will confirm whether someone has lupus.
Doctors don’t routinely screen people with lupus for PAH. If someone with lupus develops breathlessness, chest pain, lightheadedness or swollen feet, they might have lupus-associated PAH and should talk with their care team about screening.
A careful and detailed evaluation is especially important if doctors suspect lupus-associated PAH. Lupus can cause other problems in the heart and lungs. Therefore, doctors must rule out lupus-related heart inflammation and scarring in the lungs to make sure those conditions aren’t exacerbating a patient’s symptoms.
Some common tests in a PAH workup are:
- Echocardiogram
- Pulmonary function tests
- Ventilation-perfusion (V/Q) scan
- Natriuretic peptide tests
- Right heart catheterization
After testing is completed, doctors will diagnose lupus-associated PAH only if the right heart catheterization shows high blood pressure in the lungs and they can’t determine other heart or lung conditions.
Although people with lupus have a higher risk of developing PAH compared to the general population, it’s not clear whether lupus causes PAH or makes PAH more likely to develop.
Treatment options
A PH specialist will use a variety of tests to determine the severity of a patient’s PAH and base treatment decisions on the results. Follow-up testing should be used to determine the success of these strategies.
With the variety of PAH therapies available, follow-up visits and testing with a PH specialist should take place regularly so doctors can customize treatment strategies to patients’ specific disease, lifestyle and goals.
Lupus treatment also can improve blood pressure in the lungs. Most treatments are drugs that suppress the immune system and reduce the inflammation caused by lupus.
Doctors recommend that people with lupus-associated PAH go to treatment centers familiar with this complex condition.
Patients with lupus-associated PAH tend to respond well to current treatment options. However, because lupus is a complex disease that can affect other organs, treatment can be complicated. Close follow-up visits with a PH specialist and rheumatologist can ensure both diseases are well-controlled and properly treated.
Follow up testing and current treatments
In the last few years, the Food and Drug Administration has approved several new treatments for PAH. People with lupus-associated PAH can be treated with any of those therapies.
- Targeted Therapies for Pulmonary Hypertension
Targeted medications for pulmonary hypertension are the result of years of research and development, including clinical trials with patients. These treatments work by addressing chemical imbalances in the lungs.
Diagnosis & treatment
- Supportive Medical Therapies for Pulmonary Hypertension
Supportive medical therapies that may be prescribed for pulmonary hypertension include supplemental oxygen, diuretics and blood thinners among others. Caution is advised for over-the-counter medications.
Diagnosis & treatment
- Testing for Types and Severity of PH
In addition to diagnostic tests that reveal whether a person has PH, doctors may order tests that will help identify the cause of pulmonary hypertension and risk classification. These tests may also help determine the best treatment options.
Diagnosis & treatment
- Group 1: Pulmonary Arterial Hypertension
Group 1 pulmonary hypertension is also known as pulmonary arterial hypertension. PAH occurs when the arteries in the lungs narrow and stiffen, leading to increased pressure in the lungs.
About PH
- Diagnosing Pulmonary Hypertension
Common symptoms, many causes and a variety of tests can make pulmonary hypertension difficult to diagnose. Knowing what symptoms to look for and diagnostic tests that provide critical information can lead to an earlier diagnosis and better outcomes.
- Pulmonary Hypertension Care Centers
PHA-accredited Pulmonary Hypertension Care Centers improve outcomes and quality of care for people with PH. Find expert care by locating a center or specialist near you.
Additional Resources
Download or order a brochure
Information on PH and Lupus is one of a series of PH& brochures available to download as a PDF or to order as a printed brochure.
PH & Lupus
