OP0239 PROGRESSIVE LUNG FIBROSIS IN PATIENTS WITH SYSTEMIC SCLEROSIS-ASSOCIATED INTERSTITIAL LUNG DISEASE IN THE EUSTAR DATABASE
Authors: Anna-Maria Hoffmann-Vold, Yannick Allanore, Margarida Alves, Nicole Graf, Paolo Airò, Lidia P. Ananyeva, László Czirják, Serena Guiducci, Eric Hachulla, LI Mengtao, Carina Mihai, Gabriela Riemekasten, Petros Sfikakis, Gabriele Valentini, Otylia Kowal-Bielecka, Oliver Distler
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Some patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) develop progressive ILD, but the prevalence is unknown and no validated algorithms for early detection exist. <h3>Objectives</h3> To assess the frequency of, and parameters associated with, progressive SSc-ILD in the EUSTAR database. <h3>Methods</h3> Patients from the EUSTAR database registered after 2010, ≥18 yrs old, fulfilling SSc classification criteria, with serial lung function, HRCT assessments and disease duration recordings were eligible. Progressive ILD, assessed as absolute changes in%predicted, was defined as: significantly progressive (FVC decline &gt;10% or FVC decline 5−10% and DL<sub>CO</sub> decline ≥15%); moderately progressive (FVC decline 5−10%); or stable (&lt;5% FVC change in either direction). Lung fibrosis changes from baseline to 12±3 months, disease course and cumulative frequency of progressive ILD during the 5-yr follow-up were assessed. Candidate predictors of significant progressive ILD were selected by expert opinion, and logistic regression applied. Time to event was evaluated by Kaplan-Meier estimates. <h3>Results</h3> 826 SSc-ILD patients were included; 219/826 (26.5%) showed moderate/significant ILD progression at 12±3 months' follow-up. Baseline FVC, erythrocyte sedimentation rate, modified Rodnan skin score (mRSS), GERD and disease duration were significantly associated with significant progressive fibrosis in uni- and multivariable modelling (area under curve 0.67). Serial lung function data with median follow-up of 5 yrs were available in 411/826 (49.8%) patients. In 128/411 with initial progressive ILD at 12±3 months, 59/128 (46.1%) showed further progression, while 69/128 (53.9%) were stable. Baseline FVC, mRSS and disease duration were significantly associated with further ILD progression. The incident cumulative frequency of moderate and significant ILD progression was 22.9% (n=189/826) and 28.5% (n=235/826) during the median 5-yr follow-up and time to first progressive event (Figure). <h3>Conclusion</h3> This study provides novel insights into the frequency, severity and association of progressive fibrosis in patients with SSc-ILD in the EUSTAR database. <h3>Acknowledgement</h3> Funding: Boehringer Ingelheim (Schweiz) GmbH, Switzerland <h3>Disclosure of Interests</h3> Anna-Maria Hoffmann-Vold Grant/research support from: Received research funding or other remuneration from Boehringer Ingelheim, GSK, and Actelion, Consultant for: Received consulting fees or other remuneration from Boehringer Ingelheim, GSK, and Actelion, Speakers bureau: Actelion and Boehringer Ingelheim, Yannick Allanore Grant/research support from: Inventiva, F Hoffman La-Roche, Sanofi, BMS, Pfizer, Consultant for: Actelion, Bayer, BMS, Boehringer, Roche, Sanofi, Margarida Alves Employee of: Employee of Boehringer Ingelheim, Nicole Graf Grant/research support from: Received consulting fees or other remuneration from Astellas and Biotronik, Paolo Airò: None declared, Lidia P. Ananyeva: None declared, László Czirják: None declared, Serena Guiducci: None declared, Eric Hachulla Consultant for: Received consulting fees or other remuneration from Actelion, GSK, Pfizer, and Bayer, Mengtao Li: None declared, Carina Mihai Consultant for: Received consulting fees or other remuneration from Actelion, Geneva, Roche, and Rofarm, Consultant for: F. Hoffmann-La Roche, Actelion, Geneva Romfarm, Gabriela Riemekasten Consultant for: Chugai, F. Hoffmann-La Roche, Speakers bureau: Chugai, F. Hoffmann-La Roche, Petros Sfikakis: None declared, Gabriele Valentini Grant/research support from: Received research funding and other remuneration from AbbVie, BMS, Lilly, Pfizer, and Sanofi, Consultant for: Received consulting fees and other remuneration from AbbVie, BMS, Lilly, Pfizer, and Sanofi, Otylia Kowal-Bielecka Consultant for: Received consulting fees or other remuneration from Bayer, Boehringer Ingelheim, Inventiva, Medac, Novartis, and Roche, Oliver Distler Grant/research support from: Prof. Distler received research funding from Actelion, Bayer, Boehringer Ingelheim and Mitsubishi Tanabe to investigate potential treatments of scleroderma and its complications, Consultant for: Prof. Distler has/had consultancy relationship within the last 3 years with Actelion, AnaMar, Bayer, Boehringer Ingelheim, ChemomAb, espeRare foundation, Genentech/Roche, GSK, Inventiva, Italfarmaco, iQvia, Lilly, medac, MedImmune, Mitsubishi Tanabe Pharma, Pharmacyclics, Novartis, Pfizer, Sanofi, Serodapharm and UCB in the area of potential treatments of scleroderma and its complications. In addition, he had/has consultancy relationship within the last 3 years with A. Menarini, Amgen, Abbvie, GSK, Mepha, MSD, Pfizer and UCB in the field of arthritides and related disorders