TY - JOUR T1 - Predictors of progression in systemic sclerosis patients with interstitial lung disease JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.02026-2019 SP - 1902026 AU - Oliver Distler AU - Shervin Assassi AU - Vincent Cottin AU - Maurizio Cutolo AU - Sonye K. Danoff AU - Christopher P. Denton AU - Jörg H.W. Distler AU - Anna-Maria Hoffmann-Vold AU - Sindhu R. Johnson AU - Ulf Müller Ladner AU - Vanessa Smith AU - Elizabeth R. Volkmann AU - Toby M. Maher Y1 - 2020/01/01 UR - http://erj.ersjournals.com/content/early/2020/02/14/13993003.02026-2019.abstract N2 - Systemic sclerosis (SSc) is a systemic autoimmune disease affecting multiple organ systems, including the lungs. Interstitial lung disease (ILD) is the leading cause of death in SSc.There are no valid biomarkers to predict the occurrence of SSc-ILD, although auto-antibodies against anti-topoisomerase I and several inflammatory markers are candidate biomarkers that need further evaluation. Chest auscultation, presence of shortness of breath and pulmonary function testing are important diagnostic tools, but lack sensitivity to detect early ILD. Baseline screening with high-resolution computed tomography (HRCT) is therefore necessary to confirm an SSc-ILD diagnosis. Once diagnosed with SSc-ILD, patients' clinical courses are variable and difficult to predict, though certain patient characteristics and biomarkers are associated with disease progression. It is important to monitor patients with SSc-ILD for signs of disease progression, though there is no consensus about which diagnostic tools to use or how often monitoring should occur. In this article, we review methods used to define and predict disease progression in SSc-ILD.There is no valid definition of SSc-ILD disease progression, but we suggest that either a decline in forced vital capacity (FVC) from baseline of ≥10%, or an FVC decline of 5–9% in association with a decline in diffusing capacity of carbon monoxide of ≥15% represents progression. An increase in the radiographic extent of ILD on HRCT imaging would also signify progression. A time period of 1–2 years is generally used for this definition, but a decline over a longer time period may also reflect clinically relevant disease progression.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Distler reports personal fees from Boehringer, during the conduct of the study; grants and personal fees from Actelion, personal fees from Abbvie, personal fees from Acceleron Pharma, personal fees from Anamar, personal fees from Amgen, grants and personal fees from Bayer, grants and personal fees from Boehringer Ingelheim, personal fees from Catenion, personal fees from CSL Behring, personal fees from ChemomAb, personal fees from Ergonex, personal fees from GSK, personal fees from Inventiva, personal fees from Italfarmaco, personal fees from iQone, personal fees from iQvia, personal fees from Medac, personal fees from Medscape, personal fees from Menarini, personal fees from Mepha, grants and personal fees from Mitsubishi, personal fees from MSD, personal fees from Lilly, personal fees from Novartis, personal fees and non-financial support from Pfizer, personal fees from Roche, personal fees from Sanofi, personal fees from Target BioScience, personal fees from UCB, personal fees from Baecon Discovery, personal fees from Blade Therapeutics, personal fees from Curzion Pharmaceuticals, from Glenmark Pharmaceuticals, outside the submitted work; In addition, Dr. Distler has a patent US8247389, EP2331143 issued.Conflict of interest: Dr. Assassi reports non-financial support from Boehringer Ingelheim, during the conduct of the study; grants from Biogen Idec, grants from Bayer, grants and personal fees from Boehringer Ingelheim outside the submitted work.Conflict of interest: Dr. Cottin reports personal fees and non-financial support from Actelion, grants, personal fees and non-financial support from Boehringer Ingelheim, personal fees from Bayer / MSD, personal fees from Gilead, personal fees from Novartis, grants, personal fees and non-financial support from Roche, personal fees from Sanofi, personal fees from Promedior, personal fees from Celgene, personal fees from Galapagos, personal fees from Galecto, outside the submitted work.Conflict of interest: Dr. Cutolo reports grants from Actelion, grants from Boehringer Ingelheim, grants from Horizon, grants from BMS, grants from Celgene, outside the submitted work.Conflict of interest: Dr. Danoff reports grants and personal fees from Boehringer-Ingelheim, grants from Genentech/Roche, grants from Bristol Meyer Squibb, personal fees from Galapagos, personal fees from Galectic, during the conduct of the study.Conflict of interest: Dr. Denton reports personal fees from Actelion, grants and personal fees from GlaxoSmithKline, personal fees from Bayer, personal fees from Sanofi, grants and personal fees from Inventiva, personal fees from Boehringer Ingelheim, grants and personal fees from CSL Behring, grants and personal fees from Leadiant Biosciences, personal fees from Corbus, during the conduct of the study.Conflict of interest: Spectra + advisory board of Boehringer-IngelheimConflict of interest: Dr. Hoffmann-Vold reports non-financial support from Boehringer Ingelheim, during the conduct of the study; grants, personal fees and non-financial support from Boehringer Ingelheim, personal fees and non-financial support from Actelion, personal fees from Roche, outside the submitted work.Conflict of interest: Dr. Johnson reports grants from Boehringer Ingelheim, Corbus, Bayer, Roche, and the Canadian Institutes of Health Research outside the submitted work.Conflict of interest: Dr. Ladner reports personal fees from Boehringer Ingelheim, outside the submitted work.Conflict of interest: Dr. Smith reports other from Boehringer Ingelheim, during the conduct of the study; grants from Boehringer Ingelheim, other from Actelion Pharmaceuticals Ltd., other from Bayer AG, other from Hoffman-La Roche AG, other from Galapagos NV, other from Sanofi, outside the submitted work.Conflict of interest: Dr. Volkmann reports personal fees from Boehringer Ingelheim, outside the submitted work.Conflict of interest: Dr. Maher reports grants and personal fees from GSK, personal fees from Boehringer Ingelheim, grants, personal fees and non-financial support from UCB, grants and personal fees from Astra Zeneca, personal fees from Roche, personal fees from Bayer, personal fees from Prometic, other from Apellis, personal fees from Samumed, personal fees from Galapagos, personal fees from Celgene, personal fees from Indalo, personal fees from Pliant, personal fees from Blade Therapeutics, from Respivant, personal fees from Novartis, personal fees from Bristol-Myers Squibb outside the submitted work. 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