Two Drugs Slow Lung Scarring in Systemic Sclerosis


Patients with systemic sclerosis who take cyclophosphamide or mycophenolate mofitil experience a decrease in lung scarring.

Patients with systemic sclerosis who take cyclophosphamide or mycophenolate mofitil experience nearly the same decrease in lung scarring, a study shows.

Neither medication is superior in reducing lung scarring and improving skin tightness. Rheumatologists can now better understand the agents’ safety profiles and know more about how to prescribe them and monitor results to help patients live longer with better lives.

In a presentation given on Nov. 8, at the 2015 ACR/ARHP annual meeting in San Francisco, Calif., University of California-Los Angeles rheumatologist Philip Clements, MD, revealed patients with systemic sclerosis experienced equivalent improvement with interstitial lung disease when taking either cyclophosphamide or mycophenolate mofitil.

“Both medications have now been shown to be effective in slowing down the scarring of the lungs in fibrosis,” Clements said. “Hopefully, this will leave patients with less breathlessness, less new fibrosis, more functionality, and longer lives. That’s what we’d really like to see happen.”

Overall, Clements said, 70 percent of patients achieved improved vital capacity in lung function, and 70 percent with thick skin experienced skin softening and thinning for improved joint function.

All total, 142 participants were divided into two groups: (1) oral cyclophosphamide 2kg/mg/day for a year, followed by a placebo for one year and (2) oral mycophenolate mofitial in doses up to 1,500 mg BID for two years. Baseline characteristics for both groups were the same.

At study’s end, improvements in forced vital capacity were roughly the same. 


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For the transition dyspnea index, there was an increase of 2.24 with cyclophosphamide versus 1.77 with mycophenolate mofitil. With modified Rodnan skin scoring, there was a decline of 5.35 with cyclophosphamide versus 4.9 with mycophenolate mofitil.

Nearly twice as many participants taking cyclophosphamide came off the medication for intolerance versus those taking mycophenolate mofitil.

The results suggest that cyclophosphamide and mycophenolate are equally efficacious in slowing lung fibrosis, reducing breathlessness and softening thickened skin. Conversely mycophenolate is better tolerated and seems safer over long term use. The researchers plan to follow the subjects for several more years to find out if the good effects last and to see how the patients are doing after completing the study. 



Study funding was provided by National Heart, Lung, and Blood Institute – National Institutes of Health.


The Scleroderma Lung Study II (SLS II) Shows That Both Oral Cyclophosphamide (CYC) and Mycophenolate Mofitil (MMF) Are Efficacious in Treating Progressive Interstitial Lung Disease (ILD) in Patients with Systemic Sclerosis (SSc);” Nov. 8, 4:30p.m.-6p.m. 2015 ACR/ARHP.

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