ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung diseaseDeveloped 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)
- PMID: 40907995
- DOI: 10.1183/13993003.02533-2024
ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung diseaseDeveloped 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)
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.
Copyright ©The Authors 2025. Published by Elsevier BV on behalf of the European Alliance of Associations for Rheumatology (EULAR), and the European Respiratory Society.
Conflict of interest statement
Conflict of interest: K.M. Antoniou reports grants from Boehringer Ingelheim, F. Hoffmann-La Roche Ltd and Menarini, consulting fees from Boehringer Ingelheim, F. Hoffmann-La Roche Ltd and GlaxoSmithKline, support for attending meetings from Chiesi, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim, F. Hoffmann-La Roche Ltd and GlaxoSmithKline. O. Distler reports grants from Kymera, Mitsubishi Tanabe and Boehringer Ingelheim, consulting fees from 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant Sciences, Amgen, AnaMar, Area, Arxx, AstraZeneca, Blade Therapeutics, Bayer, Boehringer Ingelheim, Catalyze Capital, Corbus Pharmaceuticals, CSL Behring, EMD Serono, Galapagos, Glenmark, Gossamer, Horizon, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Nkarta Inc., Novartis, Orion, Prometheus Biosciences, Quell Therapeutics, Redxpharma, Roivant, Topadur and UCB, payment or honoraria for lectures, presentations, manuscript writing or educational events from Bayer, Boehringer Ingelheim, Janssen and Medscape, patents planned, issued or pending (mir-29 for the treatment of systemic sclerosis), leadership roles with FOREUM Foundation, ERS/EULAR Guidelines, EUSTAR, SCQM, Swiss Academy of Medical Sciences (SAMW) and the Hartmann Müller Foundation, and is co-founder of Citus AG. A-M. Gheorghiu reports grants from Foundation for Research in Rheumatology (FOREUM), consulting fees from Boehringer Ingelheim, payment or honoraria for lectures, presentations, manuscript writing or educational events from Abbvie, Boehringer Ingelheim, Novartis, Sandoz and Sobi, support for attending meetings from Ewopharma, Boehringer Ingelheim and Janssen, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim. C.C. Moor reports grants from Boehringer Ingelheim, AstraZeneca and Daiichi Sankyo, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, AstraZeneca and GSK, and leadership roles with European Respiratory Society, European Lung Foundation and Advocacy Council of the ERS. J. Vikse reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Abbvie, Boehringer Ingelheim, GSK, Janssen, Merck, Novartis, ThermoFisher and UCB, and participation on a data safety monitoring board or advisory board with Novartis. E. Bargagli reports grants from Chiesi and AstraZeneca, and payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim. Y. Allanore reports grants from Alpine Immunosciences, Merck Serono, Corvus and Medsenic, consulting fees from Boehringer Ingelheim, Abbvie, AstraZeneca, Novartis, Argenx, Horizon and Topadur, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, and support for attending meetings from Celltrion and AstraZeneca. T.J. Corte reports grants from Avalyn Pharma, Boehringer Ingelheim, Pharmaxis, Bristol Myers Squibb, 4D, Roche, Pliant, Bridge Biotherapeutics and Avalyn Therapeutics, consulting fees from Boehringer Ingelheim, Pharmaxis, Bristol Myers Squibb, Ad Alta, Roche, Pliant, Bridge Biotherapeutics, Avalyn Therapeutics, DevPro and Endeavour BioMedicine, payment or honoraria for lectures, presentations, manuscript writing or educational events from Bristol Myers Squibb, Roche and Boehringer Ingelheim, support for attending meetings from Bristol Myers Squibb and Boehringer Ingelheim, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim, Ad Alta, Bristol Myers Squibb, Roche, Pliant, Bridge Biotherapeutics, Avalyn Therapeutics, DevPro and Endeavour BioMedicine. P. Dieudé reports grants from Bristol Myers Squibb and Pfizer, consulting fees from Boehringer Ingelheim, Bristol Myers Squibb, Janssen, Abbvie and Pfizer, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, Bristol Myers Squibb, Janssen, Abbvie and Pfizer, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim, Bristol Myers Squibb and Pfizer. V. Cottin reports consulting fees from Abbvie, AstraZeneca, Avalyn, Boehringer Ingelheim, BMS/Celgene, CSL (Behring, Vifor), Ferrer/United Therapeutics, Gossamer, GSK, Liquidia, Pliant, PureTech, Roche, Roivant, Sanofi and Shionogi, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, Ferrer/United Therapeutics, Roche and Sanofi, support for attending meetings from Boehringer Ingelheim and Sanofi, participation on a data safety monitoring board or advisory board with GSK and Molecure, and a leadership role (on an adjudication committee) with Fibrogen. B.A. Fisher reports grants from Janssen, Servier, Galapagos and Celgene, consulting fees from Novartis, Roche, BMS, Janssen, UCB, Sanofi, Servier, Galapagos, AstraZeneca, Otsuka, Amgen and Kiniksa, and payment or honoraria for lectures, presentations, manuscript writing or educational events from Novartis and Servier. J.T. Giles reports consulting fees from AbbVie, Pfizer, Eli Lilly, Novartis, Merck and Sana, and participation on a data safety monitoring board or advisory board with Janssen. M. Kreuter reports consulting fees from GSK, Boehringer Ingelheim, AstraZeneca, Pliant, Roche, BMS, Trevi and Galapagos, and payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim. I.E. Lundberg reports grants from Swedish Research Council (2020-01378), Region Stockholm (ALF project), Swedish Rheumatism Association, King Gustaf V 80 Year Foundation, Heart and Lung Foundation, Anna Hedin Foundation and AstraZeneca, consulting fees from Chugai Pharmaceutical Co. Ltd, Bristol Myers Squibb, EMD Serono, Research and Development Institute, Galapagos, Argenx, Pfizer and Janssen, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, and stock or stock options with Novartis and Roche. V. Poletti reports consulting fees from Boehringer Ingelheim and ERBE, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim. B. Maurer reports grants from AbbVie, Protagen and Novartis Biomedical Research, consulting fees from Novartis, Boehringer Ingelheim, Janssen-Cilag and GSK, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, GSK, Novartis, Otsuka and MSD, support for attending meetings from Medtalk, Pfizer, Roche, Actelion, Mepha, MSD and Boehringer Ingelheim, patents planned, issued or pending (mir-29 for the treatment of systemic sclerosis), and participation on a data safety monitoring board or advisory board with Janssen and Boehringer Ingelheim. E.A. Renzoni reports grants from Boehringer Ingelheim, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim and Mundipharma, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim. U. Müller-Ladner reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim and Medac. M.E. Strek reports grants from Boehringer Ingelheim Pharmaceuticals, Inc., NIH and the Pulmonary Fibrosis Foundation, consulting fees from Boehringer Ingelheim Pharmaceuticals, Inc., payment or honoraria for lectures, presentations, manuscript writing or educational events from CHEST and Boehringer Ingelheim Pharmaceuticals, Inc., support for attending meetings from Boehringer Ingelheim Pharmaceuticals, Inc., and participation on a data safety monitoring board or advisory board with Fibrogen, Bristol Myers Squibb and Pliant Therapeutics. P. Studenic reports grants from FOREUM, Horizon/HADEA, AAL, EUREKA and Jubiläumsfond der Stadt Wien, consulting fees from AbbVie, payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca and AbbVie, and support for attending meetings from Janssen and Galapagos. T. Stamm reports grants and personal fees from Roche, consulting fees and payment or honoraria for lectures, presentations, manuscript writing or educational events from AbbVie, Roche, Sanofi, Takeda and Novartis. B. Crestani reports grants from Boehringer Ingelheim, consulting fees from BMS, Boehringer Ingelheim, Chiesi, CSL Behring, GSK and Sanofi, payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca, BMS, Boehringer Ingelheim, GSK, Novartis, Roche and Sanofi, support for attending meetings from AstraZeneca, BMS, Boehringer Ingelheim, Roche and Sanofi, participation on a data safety monitoring board or advisory board with BMS, Boehringer Ingelheim, GSK, Horizon and Sanofi, and a leadership role with Fondation du Souffle. A-M. Hoffmann-Vold reports grants from Boehringer Ingelheim and Janssen, consulting fees from Abbvie, Merck Sharp & Dohme, Arxx Therapeutics, Pliant Therapeutics, BMS, Roche, Boehringer Ingelheim, Werfen, Genentech and Janssen, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, Janssen, Medscape, Merck Sharp & Dohme, Novartis and Roche, support for attending meetings from Boehringer Ingelheim, and leadership role with the CTD-ILD ERS/EULAR and EULAR study groups on the lung in rheumatic and musculoskeletal diseases. The remaining authors have no potential conflicts of interest to disclose.
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