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Journal Articles Rheumatology Year : 2023

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

David Roofeh
Kevin Brown
  • Function : Author
Ella Kazerooni
Donald Tashkin
Shervin Assassi
Fernando Martinez
Athol Wells
Ganesh Raghu
Christopher Denton
Lorinda Chung
Anna-Maria Hoffmann-Vold
Oliver Distler
Kerri Johannson
Yannick Allanore
Eric Matteson
John Pauling
James Seibold
Elizabeth Volkmann
Simon Walsh
  • Function : Author
Chester Oddis
Eric White
Shaney Barratt
Elana Bernstein
Robyn Domsic
Paul Dellaripa
Richard Conway
Ivan Rosas
Nitin Bhatt
  • Function : Author
Vivien Hsu
Francesca Ingegnoli
Bashar Kahaleh
Puneet Garcha
Nishant Gupta
Surabhi Khanna
  • Function : Author
Peter Korsten
Celia Lin
  • Function : Author
Stephen Mathai
  • Function : Author
Vibeke Strand
Tracy Doyle
  • Function : Author
Virginia Steen
Donald Zoz
  • Function : Author
Juan Ovalles-Bonilla
Ignasi Rodriguez-Pinto
Padmanabha Shenoy
Andrew Lewandoski
  • Function : Author
Elizabeth Belloli
  • Function : Author
Vivek Nagaraja
Wen Ye
  • Function : Author
Suiyuan Huang
  • Function : Author
Toby Maher

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.

Dates and versions

hal-04164473 , version 1 (18-07-2023)

Identifiers

Cite

David Roofeh, Kevin Brown, Ella Kazerooni, Donald Tashkin, Shervin Assassi, et al.. Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease. Rheumatology, 2023, 62 (5), pp.1877-1886. ⟨10.1093/rheumatology/keac557⟩. ⟨hal-04164473⟩
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