Scleroderma Patients with Lung Conditions Have Shorter Lifespans, Need Better Therapies, Study Reports

Alice Melão, MSc avatar

by Alice Melão, MSc |

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Scleroderma and lung diseases

Scleroderma patients with lung diseases have shorter lifespans than those without pulmonary conditions, underscoring the need for better treatments for patients with lung conditions, a Colorado study reports.

Another finding was that scleroderma patients with a lung disorder have higher medical costs than those without the condition. That’s particularly true of patients with pulmonary arterial hypertension, or PAH, compared with those with an interstitial lung disease, or ILD.

The study, “All-cause Healthcare Costs and Mortality in Patients with Systemic Sclerosis with Lung Involvement,” was published in The Journal of Rheumatology.

Scleroderma is an autoimmune disease whose hallmarks are thickening and scarring of tissue. An autoimmune disorder is one in which the immune system attacks healthy tissue instead of invaders.

The tissue thickening and scarring in scleroderma can affect several organs, including the lungs. Thirty to 70 percent of patients also develop ILD, and 2 to 30 percent PAH.

To get a better grasp of patient outcomes and the economic burden of scleroderma with a lung condition, a University of Colorado Medical School team used two U.S. databases to identify patients with both disorders. They were the Truven Health MarketScan Commercial and Medicare Supplemental healthcare claims repositories.

They discovered 11,752 people whom doctors diagnosed with scleroderma between 2004 and 2013. In the same period, doctors diagnosed 1,808 scleroderma patients with ILD and 1,223 with PAH.

Researchers decided to use only patients whom doctors had followed for at least five years in their healthcare costs evaluation. They included 957 patients with scleroderma, 219 with scleroderma and ILD, and 108 with scleroderma and PAH.

Sixty-five percent of the PAH patients were hospitalized during the five years. The figures were 53 percent for ILD patients and 44 percent of scleroderma patients without a lung condition. Emergency room visits were also more frequent among ILD and PAH patients than scleroderma patients without a lung disease.

PAH patients’ costs were much higher than the costs of the other two groups during the five years. The range was $18,513 to $23,268 for patients with scleroderma only, $31,285 to $55,446 for ILD patients, and $44,454 to $63,320 for PAH patients.

Another important finding was that costs increased over time, with the highest expenses of all the patients occurring in the fifth year.

Most of the costs involved hospital admissions, outpatient services and medicine. PAH patients had the highest outpatient services and medicine expenses.

The cost that varied the most among the three patient groups was medicine. PAH patients’ cost were more than four times that of scleroderma patients without a lung condition and nearly double that of ILD patients.

Among those who were treated at least 10 years after their diagnosis, the team found that scleroderma patients with a lung condition had the worst death rates. The median survival time after diagnosis was 11.3 years for scleroderma patients with no lung disorder, 8.8 years for ILD patients and six years for PAH patients.

“In comparison to those with SSc [scleroderma] alone, the presence of ILD and/or PAH is associated with substantial increases in healthcare costs and a worse survival experience,” the team wrote.

“Given the effect of lung disease on survival with SSc, these data highlight that there remains an unmet need to identify more effective therapeutic strategies for both ILD and PAH in this patient population,” they concluded.