Therapeutic Choices in Systemic Sclerosis-Associated Interstitial Lung Disease, a Survey of 2 International Research Groups.

Background/objective: Options for systemic sclerosis-associated interstitial lung disease (SSc-ILD) have evolved rapidly. Mycophenolate mofetil (MMF) has replaced cyclophosphamide (CYC) in most cases of SSc-ILD, with the recent addition of tocilizumab (TCZ) in SSc-ILD as well. Combination immunosuppressive (CI) therapy with rituximab (RTX) and MMF, along with the antifibrotic nintedanib, have also become options. We aimed to better understand prescribing patterns and examine treatment trends overall to examine guideline penetrance.

Methods: A survey polling international SSc experts was conducted from October 2023 through March 2024 by members of the Scleroderma Clinical Trials Consortium and the European Scleroderma Trials and Research Group.

Results: MMF was the most common first-line treatment (92%) for SSc-ILD, followed by a split preference for RTX or TCZ for second/third line. Most experts add an antifibrotic (57%) or use CI therapy (24%) with failure of initial therapy. When CI therapy is used, MMF/RTX is used most (71%), followed by MMF/TCZ (38%). Corticosteroids were used for SSc-ILD treatment by 36% of experts, with 12% of these respondents using greater than 20 mg of prednisone equivalent. The survey response rate was 17.4% of total centers and 7.7% of total experts.

Conclusion: First-line treatment preferences are in line with current treatment guidelines. CI therapy is not typically used, although the EVER-ILD trial might have influenced prescribing patterns with RTX/MMF CI therapy more typical. Prednisone use was more common than expected. Further studies evaluating combination MMF/TCZ versus MMF/RTX and whether TCZ is effective for SSc-ILD in patients without an inflammatory laboratory profile are necessary to help guide clinical practice. Further adoption of current guidelines may also change prednisone use patterns.