Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease

Archive ouverte

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 | Khanna, Dinesh

Edité par CCSD ; Oxford University Press (OUP) -

International audience. 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.

Consulter en ligne

Suggestions

Du même auteur

Clinical characteristics, visceral involvement, and mortality in at-risk or early diffuse systemic sclerosis: a longitudinal analysis of an observational prospective multicenter US cohort

Archive ouverte | Jaafar, Sara | CCSD

International audience. BackgroundEarly diffuse cutaneous systemic sclerosis (dcSSc) has the highest case fatality among rheumatic diseases. We report baseline characteristics, current immunosuppressive therapies, p...

Efficacy and safety of nintedanib in patients with systemic sclerosis-associated interstitial lung disease treated with mycophenolate: a subgroup analysis of the SENSCIS trial.

Archive ouverte | Highland, Kristin | CCSD

International audience. Background: In the Safety and Efficacy of Nintedanib in Systemic Sclerosis (SENSCIS) trial, nintedanib reduced the rate of decline in forced vital capacity (FVC) in patients with systemic scl...

Diagnosis and monitoring of systemic sclerosis-associated interstitial lung disease using high-resolution computed tomography

Archive ouverte | Khanna, Dinesh | CCSD

International audience. Patients with systemic sclerosis are at high risk of developing systemic sclerosis–associated interstitial lung disease. Symptoms and outcomes of systemic sclerosis–associated interstitial lu...

Chargement des enrichissements...