ILD is frequently progressive in patients with Sjogren’s disease

<bold>Background:</bold> Limited knowledge of the disease course of ILD in primary Sjögren’s disease (SjD) exist. <bold>Objectives:</bold> Assess progressive ILD in SjD applying different definitions of progressive ILD and compare to other CTD-ILDs. <bold>Methods:</bold> 647 CTD-ILD patients including 46 (7%) SjD-ILD patients from well characterized cohorts at expert centers (Oslo and Zurich) were assessed. Progressive ILD was defined as absolute FVC decline ≥5% and ≥10% over 12 months and fulfilling the PPF criteria. In addition, absolute FVC decline over 12 months was compared between groups. <bold>Results:</bold> Patients with SjD-ILD had higher baseline FVC% compared to other CTDs, and higher but still impaired DLCO% values (Image 1). We identified in total 117 (25%) CTD-ILD patients with FVC decline ≥5%, 56 (12%) with FVC decline ≥10% and 53 (15%) patients with PPF (Image 2). Corresponding figures in pSS-ILD were 28%, 13% and 20%, showing that SjD-ILD is as progressive as other CTD-ILDs using any of the applied definitions (Image 2). Absolute FVC% decline was most pronounced in patients with SjD-ILD compared to other CTD-ILDs despite standard of care treatment (Image 1 and 2). In addition, worsening on HRCT was more pronounced in SjD-ILD than in other groups. <bold>Conclusion:</bold> In this study, progressive ILD is as frequent in SjD as in the other CTDs. This highlights the urgent need for optimized management approaches including early identification and monitoring of SjD-ILD patients. <fig><object-id>erj;64/suppl_68/PA4268/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>