High Costs, Poor Survival in Scleroderma Patients with PAH Point to Need for Better Treatments, Study Argues

Scleroderma patients with newly diagnosed pulmonary arterial hypertension (PAH) have significantly higher healthcare costs than those without PAH, a Colorado study shows.

The researchers said scleroderma patients with interstitial lung disease (ILD) also face higher costs.

Both lung conditions are associated with poorer scleroderma patient survival, demonstrating the  need for better treatments for this patient group, researchers at the University of Colorado School of Medicine argued.

Scleroderma is an autoimmune disease that damages skin, internal organs, muscles and blood vessels. An autoimmune disease is one in which the immune system attacks healthy tissue instead of invaders.

Up to 70 percent of people with scleroderma develop a lung disease. That’s because scleroderma causes the tissue scarring and blood vessel destruction that are hallmarks of lung conditions.  Importantly, lung disease is the main cause of death among scleroderma patients.

As with other chronic diseases, healthcare costs are high with scleroderma.

To get a better grasp of how a lung disease affects these costs, researchers examined them in people with newly diagnosed scleroderma and in scleroderma patients with newly diagnosed PAH or ILD.

The study, “All-cause Healthcare Costs and Mortality in Patients with Systemic Sclerosis with Lung Involvement,” appeared in The Journal of Rheumatology. Genentech and its parent company Roche funded the research.

The team used two healthcare claims databases to identify patients — the Truven Health MarketScan Commercial and Medicare Supplemental compilations. They followed them for five years.

Their study included 1,957 patients with newly diagnosed scleroderma, 219 with scleroderma and newly diagnosed ILD, and 108 with scleroderma and newly diagnosed PAH.

Because there was considerable overlap between the three groups, the team did not do a statistical analyses of their findings.

Most patients were women, as is common in scleroderma, and the most common additional medical conditions were acid reflux and high blood pressure.

Hospitalization was more common in the PAH group than the ILD and scleroderma groups over  the five years. Sixty-four percent of PAH patients and 53 percent of ILD patients had at least one admission. The figure was 44 percent of those with scleroderma without a lung disease.

Healthcare costs were highest in PAH patients — an average of $254,425 over the five years. Costs for ILD patients averaged $191,107, and for scleroderma patients with no lung disease $101,839. Costs tended to be the highest in the fifth year.

The majority of costs involved medicine, outpatient services, and hospital admissions. The biggest difference among the three groups was in medicine costs. The drug costs of those with PAH were nearly four times higher than those of scleroderma patients without a lung disease and twice that of the ILD group.

PAH-specific therapies drove the difference in costs. The average five-year cost of such drugs was more than 10 times higher in scleroderma PAH patients than in scleroderma patients without a lung disease.

Meanwhile, scleroderma PAH patients survived a median of only six years. Scleroderma ILD patients lived a median of 8.8 years, and those without a lung disease 11.3 years.

The statistics clearly showed that better treatments are needed for scleroderma patients who also develop a lung disease and that PAH is linked to particularly poor outcomes and high costs.