Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease. (29th September 2022)

• Record Type: Journal Article
• Authors: Roofeh, David; Brown, Kevin K; Kazerooni, Ella A; Tashkin, Donald; Assassi, Shervin; Martinez, Fernando; Wells, Athol U; Raghu, Ganesh; Denton, Christopher P; Chung, Lorinda; Hoffmann-Vold, Anna-Maria; Distler, Oliver; Johannson, Kerri A; Allanore, Yannick; Matteson, Eric L; Kawano-Dourado, Leticia; Pauling, John D; Seibold, James R; Volkmann, Elizabeth R; Walsh, Simon L F; Oddis, Chester V; White, Eric S; Barratt, Shaney L; Bernstein, Elana J; Domsic, Robyn T; Dellaripa, Paul F; Conway, Richard; Rosas, Ivan; Bhatt, Nitin; Hsu, Vivien; Ingegnoli, Francesca; Kahaleh, Bashar; Garcha, Puneet; Gupta, Nishant; Khanna, Surabhi; Korsten, Peter; Lin, Celia; Mathai, Stephen C; Strand, Vibeke; Doyle, Tracy J; Steen, Virginia; Zoz, Donald F; Ovalles-Bonilla, Juan; Rodriguez-Pinto, Ignasi; Shenoy, Padmanabha D; Lewandoski, Andrew; Belloli, Elizabeth; Lescoat, Alain; Nagaraja, Vivek; Ye, Wen; Huang, Suiyuan; Maher, Toby; Khanna, Dinesh;
• Is Part Of: Rheumatology. Volume 62:Number 5(2023)
• Page Start: 1877
• Page End: 1886
• Publication Date: 2022-09-29
• Subjects: systemic sclerosis interstitial lung disease -- connective tissue disease interstitial lung disease -- systemic sclerosis associated interstitial lung disease subsets; Rheumatism -- Periodicals; Rheumatology -- Periodicals; 616.723005
• DOI: 10.1093/rheumatology/keac557
• Languages: English
• ISSNs: 1462-0324

Abstract

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.