Early diagnosis of pulmonary arterial hypertension is crucial for improving patient outcomes, according researchers at the University of California at Los Angeles.

PAH is a rare disease, with 15,000-25,000 patients diagnosed and treated in the United States. Patient populations at risk for PAH include users of anorexigens or methamphetamines, patients with scleroderma, HIV infection, portal hypertension, cirrhosis or congenital heart disease, as well as family members of patients with idiopathic or heritable PAH. Symptoms of PAH include dyspnea, fatigue, syncope or near syncope, chest pain, palpitations and leg edema.

According to UCLA’s to Glenna Traiger, RN, MSN, CNS-BC, PAH is difficult to diagnose because it occurs most often among women who are overweight, so their symptoms are typically misdiagnosed at first.

“The delay in diagnosis persists even though we have had drugs for this condition since the late 1990s,” she said. “Most patients are diagnosed with late symptoms; and, when it is untreated, PAH has a very poor prognosis.”