1. Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease.
- Author
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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, and Khanna, Dinesh
- Subjects
connective tissue disease interstitial lung disease ,systemic sclerosis associated interstitial lung disease subsets ,systemic sclerosis interstitial lung disease ,Humans ,Lung Diseases ,Interstitial ,Scleroderma ,Systemic ,Vital Capacity ,Tomography ,X-Ray Computed ,Severity of Illness Index ,Lung - 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.
- Published
- 2023