OP0068 INTERSTITIAL LUNG DISEASE IS FREQUENTLY PROGRESSIVE IN PATIENTS WITH PRIMARY SJÖGREN’S SYNDROME
Authors: M. Sprecher, E. Langballe, P. P. Diep, H. Fretheim, H. Andersson, T. M. Aaløkken, C. Brunborg, C. Bruni, C. Clarenbach, M. T. Durheim, T. Frauenfelder, Ø. Molberg, O. Distler, A. M. Hoffmann-Vold
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Interstitial lung disease (ILD) in primary Sjögren's syndrome (pSS) has been reported to be present in 10-15% of patients. There are limited data on the disease course of pSS-ILD, and it is not clear how the ILD in pSS evolves compared to the ILD in other rheumatic musculoskeletal diseases (RMDs) such as systemic sclerosis (SSc), antisynthetase syndrome (ASS) and rheumatoid arthritis (RA). These knowledge gaps are important to fill for clarification whether it is appropriate or not to lump pSS-ILD together with other RMD-ILD types for clinical trial purposes, given the limited treatment options for pSS-ILD. <h3>Objectives:</h3> Assess proportion and rate of progressive ILD in pSS applying several definitions for progressive ILD and compare to other RMD-ILDs. <h3>Methods:</h3> We included pSS-ILD patients from well characterized cohorts at two expert RMD-ILD centers (Oslo and Zurich). Eligibility criteria were ILD on HRCT, consecutive annual lung function assessments including forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) and comprehensive serial clinical and imaging assessments. We assessed progressive disease in pSS-ILD and compared it to other RMD-ILDs applying the following definitions for ILD progression: (I)Absolute FVC decline ≥5% and ≥10% over 12 months (II)2022 ATS/ERS/JRS/ALAT guideline progressive pulmonary fibrosis (PPF) criteria with (1) worsening of respiratory symptoms; (2) absolute decline in FVC ≥5% or in DLCO ≥10% and (3) disease progression on HRCT, over 12 months In addition, absolute FVC decline over 12 months was compared between groups. We compared ILD progression across the diseases using descriptive statistics including ANOVA. <h3>Results:</h3> In total, 647 RMD-ILD patients met the study eligibility criteria and were enrolled in the study cohort. The total cohort included 46 (7%) patients with pSS-ILD, 127 (20%) with ASS-ILD, 79 (12%) with RA-ILD, 65 (10%) with other idiopathic inflammatory myopathies (IIM) and 24 (4%) with mixed connective tissue disease (MCTD) (Table 1). Baseline and 1 year follow-up lung function data was available in 477 and 346 patients, respectively. Patients with pSS-ILD had higher baseline FVC% compared to other RMDs, and higher but impaired DLCO% values (Table 1). In total, we identified 117 (25%) RMD-ILD patients with FVC decline ≥5%, 56 (12%) with FVC decline ≥10% and 53 (15%) patients with PPF (Table 2). Corresponding figures in pSS-ILD were 28 %, 13% and 20%, showing that pSS-ILD was as progressive as other RMD-ILDs using any of the applied definitions (Table 2). Absolute FVC% decline was most pronounced in patients with pSS-ILD compared to other RMD-ILDs despite standard of care treatment (Table 1 and 2). In addition, worsening on HRCT was more pronounced in pSS-ILD than in other groups. <h3>Conclusion:</h3> In this study, progressive ILD is as frequent in pSS as in the other RMDs assessed. This highlights the urgent need for optimized management approaches including early identification and monitoring of pSS-ILD patients. Our results indicate that it is appropriate to include patients with pSS-ILD in clinical trials with basket approaches. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> Marco Sprecher AbbVie, Emily Langballe Boehringer Ingelheim, Phuong Phuong Diep Boehringer Ingelheim, Boehringer Ingelheim, Håvard Fretheim Boehringer Ingelheim, Helena Andersson: None declared, Trond M Aaløkken Boehringer Ingelheim, Cathrine Brunborg: None declared, Cosimo Bruni Eli-Lilly Boehringer Ingelheim, Novartis Foundation for Biomedical Research, AbbVie, Wellcome Trust, Christian Clarenbach Boehringer Ingelheim, GSK, Astra Zeneca, Sanofi, Vifor, Grifols, OM Pharma, CSL Behring, Boehringer Ingelheim, GSK, Astra Zeneca, Sanofi, Vifor, Grifols, OM Pharma, Daiichi Synkyo, CSL Behring, Michael T Durheim Boehringer Ingelheim, Roche, Boehringer Ingelheim, Thomas Frauenfelder Bayer, Bracco, Boehringer Ingelheim, Bayer, Øyvind Molberg: None declared, Oliver Distler Boehringer Ingelheim, Janssen, Medscape, CITUS AG, 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Argenx, Arxx, AstraZeneca, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, Horizon, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Orion, Prometheus, Redxpharma, Roivant, Topadur, UCB., Anna-Maria Hoffmann-Vold Boehringer Ingelheim, Boehringer Ingelheim, Janssen, Medscape, Merck Sharp &amp; Dohme, Novartis, Roche, ARXX, BMS, Boehringer Ingelheim, Genentech, Janssen, Medscape, Merck Sharp &amp; Dohme, Roche, Boehringer Ingelheim, Janssen.