The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements

Hoffmann-Vold AM, Maher TM, Philpot EE, Ashrafzadeh A, Barake R, Barsotti S, Bruni C, Carducci P, Carreira PE, Castellví I, Del Galdo F, Distler J, Foeldvari I, Fraticelli P, George PM, Griffiths B, Guillén-Del-Castillo A, Hamid AM, Horváth R, Hughes M, Kreuter M, Moazedi-Fuerst F, Olas J, Paul S, Rotondo C, Rubio-Rivas M, Seferian A, Tomčík M, Uzunhan Y, Walker UA, Więsik-Szewczyk E, Distler O (2020)

Publication Type: Journal article

Publication year: 2020

Journal

Book Volume: 2

Pages Range: e71-e83

Journal Issue: 2

DOI: 10.1016/S2665-9913(19)30144-4

Abstract

Background: Systemic sclerosis-associated interstitial lung disease (ILD) carries a high mortality risk; expert guidance is required to aid early recognition and treatment. We aimed to develop the first expert consensus and define an algorithm for the identification and management of the condition through application of well established methods. Methods: Evidence-based consensus statements for systemic sclerosis-associated ILD management were established for six domains (ie, risk factors, screening, diagnosis and severity assessment, treatment initiation and options, disease progression, and treatment escalation) using a modified Delphi process based on a systematic literature analysis. A panel of 27 Europe-based pulmonologists, rheumatologists, and internists with expertise in systemic sclerosis-associated ILD participated in three rounds of online surveys, a face-to-face discussion, and a WebEx meeting, followed by two supplemental Delphi rounds, to establish consensus and define a management algorithm. Consensus was considered achieved if at least 80% of panellists indicated agreement or disagreement. Findings: Between July 1, 2018, and Aug 27, 2019, consensus agreement was reached for 52 primary statements and six supplemental statements across six domains of management, and an algorithm was defined for clinical practice use. The agreed statements most important for clinical use included: all patients with systemic sclerosis should be screened for systemic sclerosis-associated ILD using high-resolution CT; high-resolution CT is the primary tool for diagnosing ILD in systemic sclerosis; pulmonary function tests support screening and diagnosis; systemic sclerosis-associated ILD severity should be measured with more than one indicator; it is appropriate to treat all severe cases; no pharmacological treatment is an option for some patients; follow-up assessments enable identification of disease progression; progression pace, alongside disease severity, drives decisions to escalate treatment. Interpretation: Through a robust modified Delphi process developed by a diverse panel of experts, the first evidence-based consensus statements were established on guidance for the identification and medical management of systemic sclerosis-associated ILD. Funding: An unrestricted grant from Boehringer Ingelheim International.

Authors with CRIS profile

Involved external institutions

Università degli Studi di Firenze / University of Florence Italy (IT) Imperial College London / The Imperial College of Science, Technology and Medicine United Kingdom (GB) Hôpital Foch France (FR) Schön Klinik Hamburg Eilbek Germany (DE) University of Pisa / Università di Pisa (UniPi) Italy (IT) Hospital de la Santa Creu i Sant Pau Spain (ES) Azienda Ospedaliero Universitaria 'Ospedali Riuniti' di Foggia Italy (IT) Małopolskie Centrum Biotechnologii Poland (PL) Bellvitge University Hospital / Hospital Universitari de Bellvitge Spain (ES) IQVIA Holdings, Inc. United States (USA) (US) Universitätsspital Zürich (USZ) Switzerland (CH) Oslo University Hospital / Oslo Universitetssykehus Rikshospitalet Norway (NO) Motol University Hospital / Fakultní nemocnice v Motole Czech Republic (CZ) University of Leeds United Kingdom (GB) National Institute for Health and Medical Research / Institut national de la santé et de la recherche médicale (INSERM) France (FR) Université Paris-Sud France (FR) Royal Preston Hospital United Kingdom (GB) Universitätsklinikum Heidelberg Germany (DE) Centre Hospitalier de Rambouillet France (FR) Università Politecnica delle Marche (UNIVPM) / Marche Polytechnic University Italy (IT) Universitätsspital Basel Switzerland (CH) Vall d'Hebron University Hospital / Hospital Universitari Vall d'Hebron Spain (ES) Hospital Universitario 12 de Octubre Spain (ES) Royal Hallamshire Hospital United Kingdom (GB) Newcastle upon Tyne Hospitals NHS Foundation Trust United Kingdom (GB) Medizinische Universität Graz Austria (AT) Presidio Ospedaliero San Salvatore Italy (IT)

How to cite

APA:

Hoffmann-Vold, A.M., Maher, T.M., Philpot, E.E., Ashrafzadeh, A., Barake, R., Barsotti, S.,... Distler, O. (2020). The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. The Lancet Rheumatology, 2(2), e71-e83. https://doi.org/10.1016/S2665-9913(19)30144-4

MLA:

Hoffmann-Vold, Anna Maria, et al. "The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements." The Lancet Rheumatology 2.2 (2020): e71-e83.

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