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Objective 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. Methods An electronic REDCap survey was distributed to 611 general rheumatologists, 348 national and international SSc experts, 285 general respirologists and 57 ILD experts. Results 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. Conclusion 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. [ABSTRACT FROM AUTHOR]

Objective A close temporal relationship between SSc onset and cancer has been reported in anti-RNA polymerase III-positive patients. We investigated the association between cancer and other SSc autoantibodies in a national SSc registry. Methods SSc patients enrolled in the Canadian Scleroderma Research Group registry from 2004 to 2019 were characterized according to autoantibodies to centromere, topoisomerase I/Scl70, RNA polymerase III, fibrillarin, Th/To (hPOP1), PM/Scl, Ku, NOR90, Ro52/TRIM21 and U1RNP. Logistic regression was used to examine the association between a close cancer-SSc interval and autoantibody status, adjusted for age, sex, race and smoking history. Results Of 1698 SSc patients, 1481 (87%) had available autoantibody data. Cancer was diagnosed within 2, 3 and 5 years of the first non-Raynaud manifestation in 1.3%, 2.1% and 3.5% of patients. The most frequent cancers diagnosed within 2 years were breast (33%), gynaecological (19%) and haematological (14%) cancers. The risk of cancer within 2 years was increased among anti-topoisomerase I [odds ratio (OR) 3.43, 95% CI: 1.04, 10.05] and anti-U1-RNP-positive patients (OR 5.54, 95% CI: 1.16, 20.40), but not with anti-RNA polymerase III. None of the anti-fibrillarin, Th/To, PM/Scl, Ku and NOR90-positive patients had cancer within 2 years. Patients with anti-centromere or none of the tested autoantibodies had numerically lower risks of developing cancer within two years. Conclusion Synchronous cancer was rare in this large cohort of predominantly female and White SSc patients. The risk of cancer within 2 years was increased among anti-topoisomerase I and anti-U1-RNP-positive patients. Screening strategies guided by autoantibodies require further careful consideration. [ABSTRACT FROM AUTHOR]

Objective Interstitial lung disease (ILD) is a leading cause of mortality in SSc. Little is known about the benefits of immunosuppressive drugs in mild ILD. Our aim was to determine whether use of CYC or MMF was associated with an improved ILD course in patients with normal or mildly impaired lung function. Methods A retrospective cohort of SSc subjects with ILD, disease duration below seven years and no exposure to CYC or MMF prior to the baseline visit was constructed from the Canadian Scleroderma Research Group registry. Subjects were categorized as having mild ILD if baseline forced vital capacity (FVC % predicted) was >85%. The primary exposure was any use of CYC or MMF at the baseline visit. FVC at one year was compared between exposed and unexposed subjects, using multivariate linear regression. Results Out of 294 eligible SSc-ILD subjects, 116 met criteria for mild ILD. In this subgroup, mean (s. d.) disease duration was 3.7 (2.0) years. Thirteen (11.2%) subjects were exposed to CYC or MMF at baseline. The one-year FVC was higher in exposed subjects compared with unexposed subjects, by a difference of 8.49% (95% CI: 0.01–16.98%). None of the exposed subjects experienced clinically meaningful progression over two years, whereas 24.6% of unexposed subjects did. Conclusion In this real-world setting, CYC/MMF exposure at baseline was associated with higher FVC values and a lower risk of progression among subjects with mild ILD. These data suggest a window of opportunity to preserve lung function in SSc-ILD. [ABSTRACT FROM AUTHOR]