Immunosuppressive drug use in limited systemic sclerosis: an international survey

Objectives Current guidelines recommend immunosuppressive treatment for diffuse cutaneous systemic sclerosis, but are less clear on their use in limited cutaneous systemic sclerosis (lcSSc) in the absence of internal organ complications. We conducted an international survey to understand current immunosuppressive drug prescribing patterns in lcSSc. Methods An electronic REDCap survey was distributed to 756 general rheumatologists and 250 national and international SSc experts. Results A total of 139 (14%) participants responded to the survey (94% were rheumatologists); 58% of respondents reported at least sometimes using immunosuppressive drugs to treat cutaneous symptoms in lcSSc, whereas 42% rarely or never did. Conversely, only 21% reported at least sometimes prescribing immunosuppression to prevent future complications in lcSSc, whereas 79% rarely or never did. Over 70% of respondents indicated that they would likely treat lcSSc in the presence of tendon friction rubs, new areas of skin thickening, increasing modified Rodnan skin scores (mRSS), or mRSS of 15 and above. Close to half would likely prescribe immunosuppression in the presence of mRSS of 10 to 14, anti‐topoisomerase I antibodies, or anti‐RNA polymerase III antibodies. Interestingly, a majority of respondents would likely treat lcSSc in the presence of subclinical organ involvement, including subclinical myocardial, muscle, joint, and lung disease. The top first‐line drugs of choice were mycophenolate mofetil (47%), methotrexate (28%), and hydroxychloroquine (20%). Conclusion This international survey highlights significant heterogeneity among rheumatologists in the use of immunosuppressive drugs in lcSSc. High‐quality research is needed to guide and harmonize the approach to management in lcSSc.