Screening and management of subclinical interstitial lung disease in systemic sclerosis: an international survey.

Abstract

Interstitial lung disease (ILD) is the leading cause of mortality in SSc. Experts now recommend high-resolution CT (HRCT) screening in all SSc patients and treatment of subclinical ILD in SSc patients with high-risk phenotypes. We undertook an international survey to understand current screening and treatment practices in subclinical SSc-ILD. An electronic REDCap survey was distributed to 611 general rheumatologists, 348 national and international SSc experts, 285 general respirologists and 57 ILD experts. One hundred and ninety-eight participants responded to the survey, including 135 (68%) rheumatologists and 54 (27%) respirologists. Over half (59%) of respondents routinely ordered HRCTs in all newly diagnosed SSc patients, although this practice was more common in Europe (83%), the USA (68%), Asia (73%) and Latin America (100%) compared with Canada (40%) and Australia (40%). Nearly half (48%) of respondents would not treat subclinical SSc-ILD, whereas 52% would treat or consider treatment. At least 70% would likely treat subclinical ILD in the setting of diffuse SSc, anti-topoisomerase-I autoantibodies, disease duration below 18 months, ground-glass opacities, oxygen desaturation, or significant ILD progression on imaging or pulmonary function tests. The majority (67%) of respirologists would not treat subclinical ILD. MMF was the preferred first-line drug for the treatment of subclinical SSc-ILD. This international survey highlights important regional variations in SSc-ILD screening and significant heterogeneity among rheumatologists and respirologists in the treatment of subclinical SSc-ILD. High-quality research addressing these questions is needed to produce evidence-based guidelines and harmonize the approach to identification and treatment of subclinical SSc-ILD.