Publication

ERS/EULAR clinical practice guidelines for connective tissue diseases associated interstitial lung disease.

Abstract
Background: Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases (CTDs) and is associated with high morbidity and mortality. Clinical practice guidelines to standardise screening, diagnosis, treatment and follow-up for CTD-ILD are of high importance for optimised patient care. Methods: A European Respiratory Society and European Alliance of Associations for Rheumatology task force committee, composed of pulmonologists, rheumatologists, pathologists, radiologists, methodologists and patient representatives, developed recommendations based on PICO (Patients, Intervention, Comparison, Outcomes) questions with grading of the evidence according to the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology and complementary narrative questions agreed on by both societies. For both PICO and narrative questions, the Evidence to Decision framework was used to formulate the recommendations. Results: The task force committee concluded with recommendations for 25 PICO and 28 narrative questions, regarding ILD in the context of systemic sclerosis, rheumatoid arthritis (RA), idiopathic inflammatory myopathies, Sjögren disease (SjD), systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD). In four narrative questions, regarding screening and assessment of risk for ILD progression in MCTD, SjD and SLE and one PICO question regarding pirfenidone in CTD-ILD other than RA-ILD, the task force had insufficient evidence to support recommendations. Screening, diagnostic, monitoring and treatment algorithms were developed based on the recommendations and usual clinical practice. Conclusions: We provide practical guidance by evidence-based recommendations to clinicians for each of the CTDs. In many cases there is low certainty or absence of evidence and we encourage further research to fill these gaps.
Author
Antoniou, Katerina M
Distler, Oliver
Gheorghiu, Ana-Maria
Moor, Catharina C
Vikse, Jens
Bizymi, Nikoleta
Galetti, Ilaria
Brown, Graham
Bargagli, Elena
Allanore, Yannick
Corte, Tamera J
Dieudé, Philippe
Cottin, Vincent
Fisher, Benjamin A
Fabre, Aurelie
Giles, Jon T
Kreuter, Michael
Lundberg, Ingrid E
Poletti, Venerino
Maurer, Britta
Renzoni, Elisabetta A
Müller-Ladner, Ulf
Strek, Mary E
Sverzellati, Nicola
Studenic, Paul
Mohammed, Jibril
Nagavci, Blin
Stamm, Tanja
Tonia, Thomy
Crestani, Bruno
Hoffmann-Vold, Anna-Maria
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Date
2025-09-04
Type
Article
Subject
Lung diseases, interstitial, Connective tissue diseases, Practice guideline, Pulmonary medicine, Rheumatology
Collections
UHB Respiratory
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Citation
Antoniou KM, Distler O, Gheorghiu AM, Moor CC, Vikse J, Bizymi N, Galetti I, Brown G, Bargagli E, Allanore Y, Corte TJ, Dieudé P, Cottin V, Fisher BA, Fabre A, Giles JT, Kreuter M, Lundberg IE, Poletti V, Maurer B, Renzoni EA, Müller-Ladner U, Strek ME, Sverzellati N, Studenic P, Mohammed J, Nagavci B, Stamm T, Tonia T, Crestani B, Hoffmann-Vold AM. ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease developed by the task force for connective tissue disease-associated interstitial lung disease of the European Respiratory Society (ERS) and the European Alliance of Associations for Rheumatology (EULAR) Endorsed by the European Reference Network on rare respiratory diseases (ERN-LUNG). Ann Rheum Dis. 2026 Jan;85(1):22-60. doi: 10.1016/j.ard.2025.08.021. Epub 2025 Sep 4.
Journal / Source Title
Annals of the Rheumatic Diseases
DOI
10.1016/j.ard.2025.08.021
URI
https://mider.dspace7.openrepository.com/handle/20.500.14200/8727
PMID
40912974
Publisher
Elsevier
Publisher’s URL
https://ard.bmj.com/
https://www.sciencedirect.com/journal/annals-of-the-rheumatic-diseases
Publisher’s statement
Note / Copyright