Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease
David Roofeh
(1)
,
Kevin Brown
,
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
,
Leticia Kawano-Dourado
(2, 3)
,
John Pauling
,
James Seibold
,
Elizabeth Volkmann
,
Simon Walsh
,
Chester Oddis
,
Eric White
,
Shaney Barratt
,
Elana Bernstein
,
Robyn Domsic
,
Paul Dellaripa
,
Richard Conway
,
Ivan Rosas
,
Nitin Bhatt
,
Vivien Hsu
,
Francesca Ingegnoli
,
Bashar Kahaleh
,
Puneet Garcha
,
Nishant Gupta
,
Surabhi Khanna
,
Peter Korsten
,
Celia Lin
,
Stephen Mathai
,
Vibeke Strand
,
Tracy Doyle
,
Virginia Steen
,
Donald Zoz
,
Juan Ovalles-Bonilla
,
Ignasi Rodriguez-Pinto
,
Padmanabha Shenoy
,
Andrew Lewandoski
,
Elizabeth Belloli
,
Alain Lescoat
(4, 5)
,
Vivek Nagaraja
,
Wen Ye
,
Suiyuan Huang
,
Toby Maher
,
Dinesh Khanna
(1)
1
University of Michigan [Ann Arbor]
2 PHERE (UMR_S_1152 / U1152) - Physiopathologie et Epidémiologie des Maladies Respiratoires
3 USP - Universidade de São Paulo = University of São Paulo
4 Irset - Institut de recherche en santé, environnement et travail
5 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Ponchaillou]
2 PHERE (UMR_S_1152 / U1152) - Physiopathologie et Epidémiologie des Maladies Respiratoires
3 USP - Universidade de São Paulo = University of São Paulo
4 Irset - Institut de recherche en santé, environnement et travail
5 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Ponchaillou]
David Roofeh
- Function : Author
- PersonId : 1270826
- ORCID : 0000-0002-9750-5771
Kevin Brown
- Function : Author
Ella Kazerooni
- Function : Author
- PersonId : 1270827
- ORCID : 0000-0001-5859-8744
Donald Tashkin
- Function : Author
- PersonId : 1270828
- ORCID : 0000-0002-5607-4872
Shervin Assassi
- Function : Author
- PersonId : 1270829
- ORCID : 0000-0002-8059-9978
Fernando Martinez
- Function : Author
- PersonId : 1270830
- ORCID : 0000-0002-2412-3182
Athol Wells
- Function : Author
- PersonId : 1270831
- ORCID : 0000-0003-2108-6248
Ganesh Raghu
- Function : Author
- PersonId : 1270832
- ORCID : 0000-0001-7506-6643
Christopher Denton
- Function : Author
- PersonId : 1270833
- ORCID : 0000-0003-3975-8938
Lorinda Chung
- Function : Author
- PersonId : 1270834
- ORCID : 0000-0003-0072-6939
Anna-Maria Hoffmann-Vold
- Function : Author
- PersonId : 1213992
- ORCID : 0000-0001-6467-7422
Oliver Distler
- Function : Author
- PersonId : 764183
- ORCID : 0000-0002-0546-8310
Kerri Johannson
- Function : Author
- PersonId : 1270835
- ORCID : 0000-0003-1205-5511
Yannick Allanore
- Function : Author
- PersonId : 1133334
- ORCID : 0000-0002-6149-0002
Eric Matteson
- Function : Author
- PersonId : 1270836
- ORCID : 0000-0002-9866-0124
Leticia Kawano-Dourado
- Function : Author
- PersonId : 1270837
- ORCID : 0000-0003-0784-1331
John Pauling
- Function : Author
- PersonId : 1226379
- ORCID : 0000-0002-2793-2364
James Seibold
- Function : Author
- PersonId : 1270838
- ORCID : 0000-0001-5187-3141
Elizabeth Volkmann
- Function : Author
- PersonId : 1270839
- ORCID : 0000-0003-3750-6569
Simon Walsh
- Function : Author
Chester Oddis
- Function : Author
- PersonId : 1270840
- ORCID : 0000-0002-9529-3111
Eric White
- Function : Author
- PersonId : 1270841
- ORCID : 0000-0003-4060-8443
Shaney Barratt
- Function : Author
- PersonId : 1270842
- ORCID : 0000-0003-3067-7349
Elana Bernstein
- Function : Author
- PersonId : 1270843
- ORCID : 0000-0001-5560-6390
Robyn Domsic
- Function : Author
- PersonId : 1270844
- ORCID : 0000-0002-2765-0922
Paul Dellaripa
- Function : Author
- PersonId : 1270845
- ORCID : 0000-0003-0237-739X
Richard Conway
- Function : Author
- PersonId : 791575
- ORCID : 0000-0003-2538-3362
Ivan Rosas
- Function : Author
- PersonId : 1270846
- ORCID : 0000-0003-4622-618X
Nitin Bhatt
- Function : Author
Vivien Hsu
- Function : Author
- PersonId : 1270847
- ORCID : 0000-0002-9588-347X
Francesca Ingegnoli
- Function : Author
- PersonId : 1270848
- ORCID : 0000-0002-6727-1273
Bashar Kahaleh
- Function : Author
- PersonId : 1270849
- ORCID : 0000-0003-4666-0250
Puneet Garcha
- Function : Author
- PersonId : 1270850
- ORCID : 0000-0003-1099-6967
Nishant Gupta
- Function : Author
- PersonId : 1270851
- ORCID : 0000-0001-9112-1315
Surabhi Khanna
- Function : Author
Peter Korsten
- Function : Author
- PersonId : 1228378
- ORCID : 0000-0001-6065-5680
Celia Lin
- Function : Author
Stephen Mathai
- Function : Author
Vibeke Strand
- Function : Author
- PersonId : 1270852
- ORCID : 0000-0003-4978-4072
Tracy Doyle
- Function : Author
Virginia Steen
- Function : Author
- PersonId : 1270853
- ORCID : 0000-0002-6333-0133
Donald Zoz
- Function : Author
Juan Ovalles-Bonilla
- Function : Author
- PersonId : 1270854
- ORCID : 0000-0001-6300-5341
Ignasi Rodriguez-Pinto
- Function : Author
- PersonId : 1270855
- ORCID : 0000-0001-6193-6396
Padmanabha Shenoy
- Function : Author
- PersonId : 1270856
- ORCID : 0000-0002-7666-1361
Andrew Lewandoski
- Function : Author
Elizabeth Belloli
- Function : Author
Alain Lescoat
- Function : Author
- PersonId : 1270857
- ORCID : 0000-0001-5289-3312
Vivek Nagaraja
- Function : Author
- PersonId : 1270858
- ORCID : 0000-0002-6134-3387
Wen Ye
- Function : Author
Suiyuan Huang
- Function : Author
Toby Maher
- Function : Author
- PersonId : 1270859
- ORCID : 0000-0001-7192-9149
Dinesh Khanna
Connectez-vous pour contacter l'auteur
- Function : Correspondent author
- PersonId : 1081160
Connectez-vous pour contacter l'auteur
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.