Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease
David Roofeh, Kevin M. Brown, Ella A. Kazerooni, Donald P. Tashkin, Shervin Assassi, Fernando J. Martínez, Athol U. Wells, Ganesh Raghu, Christopher P. Denton, Leland W.K. Chung, Anna‐Maria Hoffmann‐Vold, Oliver Distler, Kerri A. Johannson, Yannick Allanore, Eric L. Matteson, Letícia Kawano-Dourado, John D Pauling, James R. Seibold, Elizabeth R. Volkmann, Simon Walsh, Chester V. Oddis, Eric S. White, Shaney Barratt, Elana J. Bernstein, Robyn T. Domsic, Paul F. Dellaripa, Richard Conway, Iván O. Rosas, Nitin Bhatt, Vivien Hsu, Francesca Ingegnoli, Bashar Kahaleh, Puneet Garcha, Nishant Gupta, Surabhi Khanna, Peter Korsten, Celia J. F. Lin, Stephen C. Mathai, Vibeke Strand, Tracy J. Doyle, Virginia Steen, Donald F. Zoz, J.G. Ovalles-Bonilla, Ignasi Rodríguez‐Pintó, Padmanabha Shenoy, Andrew Lewandoski, Elizabeth A. Belloli, Alain Lescoat, Vivek Nagaraja, Wen Ye, Suiyuan Huang, Toby M. Maher, Dinesh Khanna
Abstract
OBJECTIVES: To establish a framework by which experts define disease subsets in systemic sclerosis associated interstitial lung disease (SSc-ILD). METHODS: A conceptual framework for subclinical, clinical and progressive ILD was provided to 83 experts, asking them to use the framework and classify actual SSc-ILD patients. Each patient profile was designed to be classified by at least four experts in terms of severity and risk of progression at baseline; progression was based on 1-year follow-up data. A consensus was reached if ≥75% of experts agreed. Experts provided information on which items were important in determining classification. RESULTS: Forty-four experts (53%) completed the survey. Consensus was achieved on the dimensions of severity (75%, 60 of 80 profiles), risk of progression (71%, 57 of 80 profiles) and progressive ILD (60%, 24 of 40 profiles). For profiles achieving consensus, most were classified as clinical ILD (92%), low risk (54%) and stable (71%). Severity and disease progression overlapped in terms of framework items that were most influential in classifying patients (forced vital capacity, extent of lung involvement on high resolution chest CT [HRCT]); risk of progression was influenced primarily by disease duration. CONCLUSIONS: Using our proposed conceptual framework, international experts were able to achieve a consensus on classifying SSc-ILD patients along the dimensions of disease severity, risk of progression and progression over time. Experts rely on similar items when classifying disease severity and progression: a combination of spirometry and gas exchange and quantitative HRCT.