Yearly PAH Screening in Scleroderma Is Crucial

Epoprostenol requires a central intravenous line, the invasive nature of which carries an infection risk. The drug has a short half-life, which can lead to pump failure and disease worsening. Treprostinil can be infused subcutaneously and has a longer half-life, but a majority of patients experience pain at the infusion site. Inhaled lipoprost requires six to nine inhalations a day, and 10 minutes per inhalation, a process that patients find “a little cumbersome,” Dr. Huston said.

Bosentan has the advantage of oral administration but is associated with abnormal elevations on liver function tests, and it interacts with several common drugs. It is contraindicated with cyclosporine and glyburide, for example. Sildenafil, called Revatio instead of Viagra when used to treat PAH, can potentiate hypotension when used with nitroglycerin. It also interacts with bosentan.

It is not clear which agent is best, or if combination therapy might help patients further, said Dr. Huston, who added he had no financial conflicts of interest to disclose.