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.
Systemic Sclerosis Associated Interstitial Lung Disease: A Conceptual Framework for Subclinical, Clinical, and Progressive Disease / D. Roofeh, K.K. Brown, E.A. Kazerooni, D. Tashkin, S. Assassi, F. Martinez, A.U. Wells, G. Raghu, C.P. Denton, L. Chung, A. Hoffmann-Vold, O. Distler, K.A. Johannson, Y. Allanore, E.L. Matteson, L. Kawano-Dourado, J.D. Pauling, J.R. Seibold, E.R. Volkmann, S.L.F. Walsh, C.V. Oddis, E.S. White, S.L. Barratt, E.J. Bernstein, R.T. Domsic, P.F. Dellaripa, R. Conway, I. Rosas, N. Bhatt, V. Hsu, F. Ingegnoli, B. Kahaleh, P. Garcha, N. Gupta, S. Khanna, P. Korsten, C. Lin, S.C. Mathai, V. Strand, T.J. Doyle, V. Steen, D.F. Zoz, J. Ovalles-Bonilla, I. Rodriguez-Pinto, P.D. Shenoy, A. Lewandoski, E. Belloli, A. Lescoat, V. Nagaraja, W. Ye, S. Huang, T. Maher, D. Khanna. - In: RHEUMATOLOGY. - ISSN 1462-0324. - (2022), pp. keac557.1-keac557.10. [Epub ahead of print] [10.1093/rheumatology/keac557]
Systemic Sclerosis Associated Interstitial Lung Disease: A Conceptual Framework for Subclinical, Clinical, and Progressive Disease
F. Ingegnoli;
2022
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.File | Dimensione | Formato | |
---|---|---|---|
Systemic sclerosis associated interstitial lung.pdf
accesso riservato
Descrizione: Original Article
Tipologia:
Publisher's version/PDF
Dimensione
2.44 MB
Formato
Adobe PDF
|
2.44 MB | Adobe PDF | Visualizza/Apri Richiedi una copia |
Pubblicazioni consigliate
I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.