Roofeh, David;
Brown, Kevin K;
Kazerooni, Ella A;
Tashkin, Donald;
Assassi, Shervin;
Martinez, Fernando;
Wells, Athol U;
... Khanna, Dinesh; + view all
(2023)
Systemic Sclerosis Associated Interstitial Lung Disease: A Conceptual Framework for Subclinical, Clinical, and Progressive Disease.
Rheumatology
, 62
(5)
pp. 1877-1886.
10.1093/rheumatology/keac557.
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Abstract
OBJECTIVES: 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 eighty-three experts, asking them to use the framework and classify actual SSc-ILD patients. Each patient profile was designed to be classified by at least 4 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.
| Type: | Article |
|---|---|
| Title: | Systemic Sclerosis Associated Interstitial Lung Disease: A Conceptual Framework for Subclinical, Clinical, and Progressive Disease |
| Location: | England |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1093/rheumatology/keac557 |
| Publisher version: | https://doi.org/10.1093/rheumatology/keac557 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | systemic sclerosis interstitial lung disease, connective tissue disease interstitial lung disease, systemic sclerosis associated interstitial lung disease subsets |
| UCL classification: | UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10156825 |
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