Patients with scleroderma at highest risk for pulmonary arterial hypertension

CHICAGO — Risks for pulmonary hypertension and pulmonary arterial hypertension among patients with scleroderma were discussed by Harrison Farber, MD, at the American College of Rheumatology State-of-the-Art Clinical Symposium, here.

Farber said patients with scleroderma are at the highest risk for developing pulmonary arterial hypertension (PAH), with approximately 60% of patients enrolled in PAH clinical trials positive for some form of scleroderma. The rate of PAH in systemic lupus erythematosus (SLE) is about 17%, and the incidence is very low in other rheumatic diseases, according to Farber.

Echocardiogram is very sensitive to detecting PAH; however, false-positives are common, and catheterization is a more accurate method of detection, Farber said.

“As we know, the [American College of Rheumatology] recommends that anybody who is newly diagnosed with scleroderma and / or is diagnosed should have an [echocardiogram], or sooner if their symptoms change,” Farber said.

According to Farber, the good news for patients with PAH is that they tend to have longer life expectancy than in the past.

“The duration of disease has increased substantially over the last few decades,” he said.

Although there is progress, Farber said PAH and interstitial lung disease (ILD) are still the leading causes of death in patients with scleroderma.

“People with PAH and scleroderma do much worse than patients with idiopathic disease or almost any of the other subgroups,” Farber said. “They are much more likely to die from their disease.”

Additional risk factors include increasing age, pericardial effusion and liver disease. Furthermore, patients with scleroderma who do not present pulmonary disease tend to have good outcomes, and the prognosis is better with scleroderma and ILD, he said.

Although recent studies have shown most patients with PAH and scleroderma have better outcomes, Farber said diagnosis can still take 2 years. Some may not present with clinical symptoms but have poorer scores on the 6-minute walk test, he said, and some may not receive the most effective treatments.

“If you look at the way [these patients] are treated, they are less frequently put on prostacyclin, which is considered the most-potent classification of drugs we can use, and ... although they don’t typically respond to calcium channel blockers, typically, they are taking them,” Farber said, adding that earlier intervention with prostacyclin therapy can lead to better outcomes.

Farber said study findings will be published later in the year from a trial with patients with PAH sponsored by the NIH in which he has been involved. The trial explores rituximab in patients with PAH who receive background therapies. - by Shirley Pulawski

Reference:

Farber H. Session IV, presentation #1. Presented at: American College of Rheumatology State-of-the-Art Clinical Symposium. May 2-3, 2015; Chicago.

Disclosure: Farber reports no relevant financial disclosures.