Pulmonary arterial hypertension can often become apparent during exercise testing in patients with systemic sclerosis, according to a report in the July issue of Chest.

Pulmonary arterial hypertension (PAH) can often become apparent during exercise testing in patients with systemic sclerosis, according to a report in the July issue of Chest.

"Pulmonary hypertension in scleroderma is a very serious and deadly complication," Dr. Virginia Steen from Georgetown University, Washington, DC told Reuters Health.

"These patients have not responded to treatment as well as other PAH patients, but we have the opportunity to identify patients earlier and get them treated before serious damage to the heart has occurred."

Dr. Steen and colleagues performed exercise echocardiograms in 54 scleroderma patients considered to be at risk for pulmonary hypertension because of dyspnea on exertion, impaired lung diffusing capacity, or high right ventricular systolic pressure (RVSP) at rest.

More than 40 per cent of the patients had a positive exercise test (where the RVSP increased by more than 20 mm Hg), the authors report.

Twenty-three patients at very high risk for PAH had a greater mean change in RVSP with exercise compared to those without high-risk pulmonary function test results, the researchers note, even though the resting RVSP did not differ significantly.

Seventeen of 21 patients with positive exercise echocardiograms who underwent right-heart catheterization had definite pulmonary hypertension, three had exercise-induced PAH, and only one had a normal right-heart catheterization result.

"It is extremely important that these high risk patients (limited scleroderma with long-standing disease, patients with anti-centromere antibody, or a nucleolar pattern on ANA testing) have yearly pulmonary function tests and echocardiograms," Dr. Steen said.

If they do have pulmonary function changes "or an increased pulmonary artery systolic pressure on echo (>35 mmHg) or have exercise-induced dyspnea, then an exercise echo should be strongly considered," she advised.

"We now have many approved treatments for pulmonary hypertension, and it is imperative that we identify and treat all patients who could benefit from this treatment," Dr. Steen concluded.

Chest 2008;134:146-151