- Roofeh, David;
- Brown, Kevin;
- Kazerooni, Ella;
- Tashkin, Donald;
- Assassi, Shervin;
- Martinez, Fernando;
- Wells, Athol;
- Raghu, Ganesh;
- Denton, Christopher;
- Chung, Lorinda;
- Hoffmann-Vold, Anna-Maria;
- Distler, Oliver;
- Johannson, Kerri;
- Allanore, Yannick;
- Matteson, Eric;
- Kawano-Dourado, Leticia;
- Pauling, John;
- Seibold, James;
- Volkmann, Elizabeth;
- Walsh, Simon;
- Oddis, Chester;
- White, Eric;
- Barratt, Shaney;
- Bernstein, Elana;
- Domsic, Robyn;
- Dellaripa, Paul;
- Conway, Richard;
- Rosas, Ivan;
- Bhatt, Nitin;
- Hsu, Vivien;
- Ingegnoli, Francesca;
- Kahaleh, Bashar;
- Garcha, Puneet;
- Gupta, Nishant;
- Khanna, Surabhi;
- Korsten, Peter;
- Lin, Celia;
- Mathai, Stephen;
- Strand, Vibeke;
- Doyle, Tracy;
- Steen, Virginia;
- Zoz, Donald;
- Ovalles-Bonilla, Juan;
- Rodriguez-Pinto, Ignasi;
- Shenoy, Padmanabha;
- Lewandoski, Andrew;
- Belloli, Elizabeth;
- Lescoat, Alain;
- Nagaraja, Vivek;
- Ye, Wen;
- Huang, Suiyuan;
- Maher, Toby;
- Khanna, Dinesh
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.