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

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

doi:10.1093/rheumatology/keac557

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

Rheumatology (Oxford). 2023 May 2;62(5):1877-1886. doi: 10.1093/rheumatology/keac557.

Authors

David Roofeh¹, Kevin K Brown², Ella A Kazerooni^{1

3}, Donald Tashkin⁴, Shervin Assassi⁵, Fernando Martinez⁶, Athol U Wells⁷, Ganesh Raghu⁸, Christopher P Denton⁹, Lorinda Chung¹⁰, Anna-Maria Hoffmann-Vold¹¹, Oliver Distler¹², Kerri A Johannson¹³, Yannick Allanore¹⁴, Eric L Matteson¹⁵, Leticia Kawano-Dourado^{16

17

18}, John D Pauling^{19

20}, James R Seibold²¹, Elizabeth R Volkmann²², Simon L F Walsh²³, Chester V Oddis²⁴, Eric S White²⁵, Shaney L Barratt^{26

27}, Elana J Bernstein²⁸, Robyn T Domsic²⁹, Paul F Dellaripa³⁰, Richard Conway³¹, Ivan Rosas³², Nitin Bhatt³³, Vivien Hsu³⁴, Francesca Ingegnoli³⁵, Bashar Kahaleh³⁶, Puneet Garcha³², Nishant Gupta³⁷, Surabhi Khanna³⁸, Peter Korsten³⁹, Celia Lin⁴⁰, Stephen C Mathai⁴¹, Vibeke Strand⁴², Tracy J Doyle⁴³, Virginia Steen⁴⁴, Donald F Zoz²⁵, Juan Ovalles-Bonilla⁴⁵, Ignasi Rodriguez-Pinto⁴⁶, Padmanabha D Shenoy⁴⁷, Andrew Lewandoski⁴⁸, Elizabeth Belloli⁴⁹, Alain Lescoat^{1

50

51}, Vivek Nagaraja¹, Wen Ye⁵², Suiyuan Huang⁵², Toby Maher⁵³, Dinesh Khanna¹

Affiliations

¹ Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, Ann Arbor, MI, USA.
² Department of Medicine, National Jewish Health, Denver, CO, USA.
³ Department of Radiology, Division of Cardiothoracic Radiology, University of Michigan, Ann Arbor, MI, USA.
⁴ Department of Medicine, Division of Pulmonary and Critical Care Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
⁵ Department of Internal Medicine, Division of Rheumatology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, USA.
⁶ Department of Internal Medicine, Division of Pulmonary Critical Care Medicine, Weill Cornell School of Medicine, New York, NY, USA.
⁷ Department of Internal Medicine, Division of Pulmonology, Royal Brompton Hospital and National Heart and Lung Institute, London, UK.
⁸ Department of Internal Medicine, Division of Pulmonology, Critical Care and Sleep Medicine, University of Washington School of Medicine, Seattle, WA, USA.
⁹ Centre for Rheumatology, Division of Medicine, University College London, London, UK.
¹⁰ Department of Internal Medicine, Division of Immunology and Rheumatology, Stanford University, and Palo Alto VA Health Care System, Palo Alto, CA, USA.
¹¹ Department of Rheumatology, Oslo University Hospital, Oslo, Norway.
¹² Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
¹³ Departments of Medicine and Community Health Sciences, Section of Respiratory Medicine, University of Calgary, Calgary, Canada.
¹⁴ Department of Rheumatology A, Cochin Hospital, APHP, Université de Paris, Paris, France.
¹⁵ Department of Internal Medicine, Division of Rheumatology, Mayo Clinic, Rochester, MN, USA.
¹⁶ HCor Research Institute, Hospital do Coração, São Paulo, Brazil.
¹⁷ Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, São Paulo, Brazil.
¹⁸ INSERM 1152, University of Paris, Paris, France.
¹⁹ Musculoskeletal Research Unit, Bristol Medical School, University of Bristol, Bristol, UK.
²⁰ Department of Rheumatology, North Bristol NHS Trust, Southmead, Bristol, UK.
²¹ Scleroderma Research Consultants, Aiken, SC, USA.
²² Department of Internal Medicine, Division of Rheumatology, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
²³ National Heart and Lung Institute, Imperial College London, London, UK.
²⁴ Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
²⁵ Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT, USA.
²⁶ Academic Respiratory Unit, School of Clinical Sciences, University of Bristol, Bristol, UK.
²⁷ Bristol Interstitial Lung Disease Service, North Bristol NHS Trust, Southmead, Bristol, UK.
²⁸ Department of Internal Medicine, Division of Rheumatology, Columbia University School of Medicine, Vagelos College of Physicians and Surgeons, New York, NY, USA.
²⁹ Department of Internal Medicine, Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
³⁰ Department of Medicine, Division of Rheumatology, Inflammation, and Immunity, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
³¹ Department of Internal Medicine, Division of Rheumatology, Trinity College Dublin, University of Dublin, Dublin, Ireland.
³² Department of Internal Medicine, Division of Pulmonology, Critical Care and Sleep Medicine, Baylor College of Medicine, Houston, TX, USA.
³³ Department of Internal Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
³⁴ Department of Internal Medicine, Division of Rheumatology, Rutgers-Robert Wood Johnson Medical School, New Brunswick, NJ, USA.
³⁵ Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Università degli Studi di Milano, Milano, Italy.
³⁶ Department of Internal Medicine, Division of Rheumatology, University of Toledo Medical Center, Toledo, OH, USA.
³⁷ Department of Internal Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of Cincinnati, Cincinnati, OH, USA.
³⁸ Department of Internal Medicine, Division of Rheumatology, University of Cincinnati, Cincinnati, OH, USA.
³⁹ Department of Nephrology and Rheumatology, University Medical Center Göttingen, Göttingen, Germany.
⁴⁰ Genentech, Inc, San Francisco, CA, USA.
⁴¹ Department of Internal Medicine, Division of Pulmonology, Critical Care and Sleep Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
⁴² Department of Internal Medicine, Division of Immunology and Rheumatology, Stanford University, Palo Alto, CA, USA.
⁴³ Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
⁴⁴ Department of Internal Medicine, Division of Rheumatology, Georgetown University School of Medicine, Washington, DC, USA.
⁴⁵ Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
⁴⁶ Autoimmune Disease Unit. Deaprtment of Internal Medicine. Hospital Mutua de Terrassa, University of Barcelona, Barcelona, Spain.
⁴⁷ Department of Rheumatology, Center for Arthritis and Rheumatism Excellence, Kochi, Kerala, India.
⁴⁸ Department of Internal Medicine, Division of Rheumatology, University of Michigan-Metro Health, Grand Rapids, MI, USA.
⁴⁹ Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor, MI, USA.
⁵⁰ Department of Internal Medicine and Clinical Immunology, Rennes University Hospital, Rennes, France.
⁵¹ Univ Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de Recherche en Santé, Environnement et Travail) - UMR_S 1085, Rennes, France.
⁵² Department of Biostatistics, School of Public Health, University of Michigan, Ann Arbor, MI, USA.
⁵³ Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, University of Southern California, Los Angeles, CA, USA.

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.

Keywords: connective tissue disease interstitial lung disease; systemic sclerosis associated interstitial lung disease subsets; systemic sclerosis interstitial lung disease.

Publication types

Research Support, N.I.H., Extramural

MeSH terms

Humans
Lung
Lung Diseases, Interstitial* / complications
Scleroderma, Systemic* / complications
Severity of Illness Index
Tomography, X-Ray Computed / methods
Vital Capacity

Abstract

Publication types

MeSH terms

Grants and funding