Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease.
Rheumatology (Oxford)
; 62(5): 1877-1886, 2023 05 02.
Article
em En
| MEDLINE
| ID: mdl-36173318
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.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Escleroderma Sistêmico
/
Doenças Pulmonares Intersticiais
Tipo de estudo:
Risk_factors_studies
Limite:
Humans
Idioma:
En
Ano de publicação:
2023
Tipo de documento:
Article