CHICAGO—About 30 years ago, pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) began to outpace renal crisis as the main causes of death in scleroderma (SSc). But treating these lung complications has proved vexing for clinicians.
There is no easy way to predict who will develop PAH. There is no telltale antibody and no clear pattern of SSc symptoms that presage the condition, so screening is important, said Janet Pope, MD, MPH, chair of rheumatology at St. Joseph’s Health Care in London, Ontario, Canada.
“There’s a chance of us preventing worsening and helping the patients [with] this lethal complication—we can prolong survival,” Dr. Pope said. “We can’t predict who will develop PAH, and it is common in scleroderma, occurring in approximately 15% of patients. That’s why we screen.”