FRI0325 IDENTIFYING SYSTEMIC SCLEROSIS PATIENTS AT RISK OF PROGRESSIVE LUNG FIBROSIS – A EUSTAR DATABASE ANALYSIS
Authors: Anna-Maria Hoffmann-Vold, Martina Gahlemann, 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, Yannick Allanore, Oliver Distler
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Early identification of patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) at risk of progression could help tailor management and aid cohort enrichment in clinical trials to improve outcome. <h3>Objectives</h3> To assess the frequency of progressive SSc-ILD in the total ILD cohort and in subgroups enriched by risk factors 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 and HRCT assessments were eligible. The following risk factors for progressive SSc-ILD available in the EUSTAR database were chosen as enrichment criteria: low forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DL<sub>CO</sub>), diffuse cutaneous SSc, anti-topoisomerase I antibody (ATA), short disease duration, older age, male sex, increased C-reactive protein, and presence of respiratory symptoms and reflux disease. Eligible patients with progressive ILD were assessed in the cohort and in subgroups enriched by the risk factors. Progressive SSc-ILD was assessed as absolute changes in%predicted and defined as: significant progressive (FVC decline &gt;10%, or FVC decline 5−10% and DL<sub>CO</sub> decline ≥15%), moderate progressive (FVC decline 5−10%) or stable (&lt;5% FVC change in either direction). The follow-up period was defined as time from baseline to last available lung function test. <h3>Results</h3> 6004 patients fulfilled the main entry criteria; of these, 1822 (30.3%) had ILD. At baseline, mean age was 57 yrs, 17.4% were male, 48.2% had diffuse SSc, 53.5% were ATA-positive, mean baseline FVC 86% and DL<sub>CO</sub>59%, mean disease duration 9.8 yrs, and follow-up was 2.5 yrs. In the total ILD cohort (30% eligible patients), 21% showed progressive ILD. Enriching with single risk factors reduced the number of eligible patients down to 6%, while the frequency of progressive ILD stayed stable (Figure). Combinations of risk factors did not result in increasing numbers of progressive ILD patients, but in further decreasing numbers of eligible patients. <h3>Conclusion</h3> There is still an unmet need to identify SSc-ILD patients at risk of progression with efficient risk factors. For clinical trials, it is a challenge to balance feasibility of recruitment and enrichment. <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, Martina Gahlemann 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, Yannick Allanore Grant/research support from: Inventiva, F Hoffman La-Roche, Sanofi, BMS, Pfizer, Consultant for: Actelion, Bayer, BMS, Boehringer, Roche, Sanofi, 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