Practice Guideline

. 2026 Jan 22;67(1):2402533.

doi: 10.1183/13993003.02533-2024. Print 2026 Jan.

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

Katerina M Antoniou^{1

2}, Oliver Distler^{3

2}, Ana-Maria Gheorghiu⁴, Catharina C Moor⁵, Jens Vikse^{6

7

8}, Nikoleta Bizymi¹, Ilaria Galetti⁹, Graham Brown^{10

11}, Elena Bargagli¹², Yannick Allanore¹³, Tamera J Corte¹⁴, Philippe Dieudé¹⁵, Vincent Cottin¹⁶, Benjamin A Fisher^{17

18}, Aurelie Fabre^{19

20}, Jon T Giles²¹, Michael Kreuter²², Ingrid E Lundberg^{23

24}, Venerino Poletti²⁵, Britta Maurer²⁶, Elisabetta A Renzoni²⁷, Ulf Müller-Ladner²⁸, Mary E Strek²⁹, Nicola Sverzellati³⁰, Paul Studenic^{23

31}, Jibril Mohammed³², Blin Nagavci³³, Tanja Stamm³⁴, Thomy Tonia³⁵, Bruno Crestani^{36

2}, Anna-Maria Hoffmann-Vold^{37

38

2}

Affiliations

¹ Department of Respiratory Medicine, Laboratory of Molecular and Cellular Pneumonology, Medical School, University of Crete, Heraklion, Greece.
² Equally contributing authors.
³ Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
⁴ Internal Medicine and Rheumatology Department, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
⁵ Centre for Interstitial Lung Diseases and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC, University Medical Centre Rotterdam, Rotterdam, The Netherlands.
⁶ Department of Clinical Science, University of Bergen, Bergen, Norway.
⁷ Department of Rheumatology, Stavanger University Hospital, Stavanger, Norway.
⁸ Department of Chemistry, Bioscience and Environmental Engineering, University of Stavanger, Stavanger, Norway.
⁹ PARE/EULAR.
¹⁰ ELF/EU-IPFF.
¹¹ Deceased.
¹² Respiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy.
¹³ Université Paris Cité, AP-HP, Hôpital Cochin, Rheumatology, Paris, France.
¹⁴ Department of Respiratory Medicine, Royal Prince Alfred Hospital and University of Sydney, Sydney, Australia.
¹⁵ Université Paris Cité, AP-HP, Hôpital Bichat-Claude Bernard, Rheumatology, Paris, France.
¹⁶ Department of Respiratory Medicine, National Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, UMR 754, INRAE, ERN-LUNG, Claude Bernard University Lyon 1, Lyon, France.
¹⁷ Rheumatology Research Group, Institute of Inflammation and Ageing, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.
¹⁸ National Institute for Health Research Birmingham Biomedical Research Centre and Department of Rheumatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
¹⁹ Department of Pathology, St Vincent's University Hospital, Dublin, Ireland.
²⁰ School of Medicine, University College Dublin, Dublin, Ireland.
²¹ Division of Rheumatology, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
²² Mainz Center for Pulmonary Medicine, Department of Pneumology, ZfT, Mainz University Medical Center and Department of Pulmonary, Critical Care and Sleep Medicine, Marienhaus Clinic Mainz, Mainz, Germany.
²³ Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden.
²⁴ Department of Gastro, Dermatology and Rheumatology, Theme Inflammation and Aging, Karolinska University, Stockholm, Sweden.
²⁵ DIMEC-Bologna University/University Morgagni Hospital Medical Specialities Department, Forlì, Italy.
²⁶ Department of Rheumatology and Immunology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
²⁷ Interstitial Lung Disease Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK.
²⁸ Department of Rheumatology and Clinical Immunology, Justus-Liebig-University Giessen, Campus Kerckhoff, Bad Nauheim, Germany.
²⁹ Section of Pulmonary and Critical Care Medicine, Department of Medicine, University of Chicago, Chicago, IL, USA.
³⁰ Scienze Radiologiche, Department of Medicine and Surgery, University of Parma, Parma, Italy.
³¹ Medical University of Vienna, Department of Internal Medicine 3, Division of Rheumatology, Wien, Austria.
³² Department of Physiotherapy, Bayero University Kano and Aminu-Kano Teaching Hospital, Kano, Nigeria.
³³ Institute for Evidence in Medicine, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg im Breisgau, Germany.
³⁴ Medical University of Vienna, Section for Outcomes Research, Center for Medical Statistics, Informatics, and Intelligent Systems, Wien, Austria.
³⁵ Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
³⁶ Université Paris Cité, AP-HP, Hôpital Bichat-Claude Bernard, Respiratory Medicine, Paris, France.
³⁷ Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland a.m.hoffmann-vold@medisin.uio.no.
³⁸ Department of Rheumatology, Oslo University Hospital, Oslo, Norway.

PMID: 40907995
PMCID: PMC12824641
DOI: 10.1183/13993003.02533-2024

Practice Guideline

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

Katerina M Antoniou et al. Eur Respir J. 2026.

. 2026 Jan 22;67(1):2402533.

doi: 10.1183/13993003.02533-2024. Print 2026 Jan.

Authors

Affiliations

¹ Department of Respiratory Medicine, Laboratory of Molecular and Cellular Pneumonology, Medical School, University of Crete, Heraklion, Greece.
² Equally contributing authors.
³ Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
⁴ Internal Medicine and Rheumatology Department, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
⁵ Centre for Interstitial Lung Diseases and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC, University Medical Centre Rotterdam, Rotterdam, The Netherlands.
⁶ Department of Clinical Science, University of Bergen, Bergen, Norway.
⁷ Department of Rheumatology, Stavanger University Hospital, Stavanger, Norway.
⁸ Department of Chemistry, Bioscience and Environmental Engineering, University of Stavanger, Stavanger, Norway.
⁹ PARE/EULAR.
¹⁰ ELF/EU-IPFF.
¹¹ Deceased.
¹² Respiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy.
¹³ Université Paris Cité, AP-HP, Hôpital Cochin, Rheumatology, Paris, France.
¹⁴ Department of Respiratory Medicine, Royal Prince Alfred Hospital and University of Sydney, Sydney, Australia.
¹⁵ Université Paris Cité, AP-HP, Hôpital Bichat-Claude Bernard, Rheumatology, Paris, France.
¹⁶ Department of Respiratory Medicine, National Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, UMR 754, INRAE, ERN-LUNG, Claude Bernard University Lyon 1, Lyon, France.
¹⁷ Rheumatology Research Group, Institute of Inflammation and Ageing, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.
¹⁸ National Institute for Health Research Birmingham Biomedical Research Centre and Department of Rheumatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
¹⁹ Department of Pathology, St Vincent's University Hospital, Dublin, Ireland.
²⁰ School of Medicine, University College Dublin, Dublin, Ireland.
²¹ Division of Rheumatology, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
²² Mainz Center for Pulmonary Medicine, Department of Pneumology, ZfT, Mainz University Medical Center and Department of Pulmonary, Critical Care and Sleep Medicine, Marienhaus Clinic Mainz, Mainz, Germany.
²³ Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden.
²⁴ Department of Gastro, Dermatology and Rheumatology, Theme Inflammation and Aging, Karolinska University, Stockholm, Sweden.
²⁵ DIMEC-Bologna University/University Morgagni Hospital Medical Specialities Department, Forlì, Italy.
²⁶ Department of Rheumatology and Immunology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
²⁷ Interstitial Lung Disease Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK.
²⁸ Department of Rheumatology and Clinical Immunology, Justus-Liebig-University Giessen, Campus Kerckhoff, Bad Nauheim, Germany.
²⁹ Section of Pulmonary and Critical Care Medicine, Department of Medicine, University of Chicago, Chicago, IL, USA.
³⁰ Scienze Radiologiche, Department of Medicine and Surgery, University of Parma, Parma, Italy.
³¹ Medical University of Vienna, Department of Internal Medicine 3, Division of Rheumatology, Wien, Austria.
³² Department of Physiotherapy, Bayero University Kano and Aminu-Kano Teaching Hospital, Kano, Nigeria.
³³ Institute for Evidence in Medicine, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg im Breisgau, Germany.
³⁴ Medical University of Vienna, Section for Outcomes Research, Center for Medical Statistics, Informatics, and Intelligent Systems, Wien, Austria.
³⁵ Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
³⁶ Université Paris Cité, AP-HP, Hôpital Bichat-Claude Bernard, Respiratory Medicine, Paris, France.
³⁷ Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland a.m.hoffmann-vold@medisin.uio.no.
³⁸ Department of Rheumatology, Oslo University Hospital, Oslo, Norway.

PMID: 40907995
PMCID: PMC12824641
DOI: 10.1183/13993003.02533-2024

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.

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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.

Figures

**FIGURE 1**
A screening algorithm for interstitial lung disease (ILD) detection in connective tissue disease (CTD) and rheumatoid arthritis (RA) based on the identification of risk factors. SSc: systemic sclerosis; MCTD: mixed connective tissue disease; IIM: idiopathic inflammatory myopathies; SjD: Sjögren disease; HRCT: high-resolution computed tomography; FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide. ^#: IIM: anti-synthetase syndrome, clinical amyopathic dermatomyositis, presence of mechanic's hands, arthritis, anti-synthetase, anti-melanoma differentiation-associated gene 5 and anti-Ro52 antibodies. ^¶: IIM: except patients with inclusion body myositis. ⁺: RA: older age, smoking history, elevated rheumatoid factor, anti-cyclic citrullinated peptide antibodies, increased inflammatory markers, male sex and high RA disease activity. ^§: SjD: older age, active extrapulmonary organ involvement and increased inflammatory markers.

**FIGURE 2**
Work-up at diagnosis algorithm for interstitial lung disease (ILD) in connective tissue disease (CTD) and rheumatoid arthritis (RA). SSc: systemic sclerosis; IIM: idiopathic inflammatory myopathies; SjD: Sjögren disease; MCTD: mixed connective tissue disease; SLE: systemic lupus erythematosus; BAL: bronchoalveolar lavage; FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; 6MWT: 6-min walk test; MALT lymphoma: mucosa-associated lymphoid tissue lymphoma. ^#: see table 4 for risk factors.

**FIGURE 3a**
Monitoring approach for patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD). FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; 6MWD: 6-min walk distance.

**FIGURE 3b**
Monitoring approach for patients with rheumatoid arthritis (RA)-associated interstitial lung disease (ILD). FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; 6MWD: 6-min walk distance.

**FIGURE 3c**
Monitoring approach for patients with interstitial lung disease (ILD) associated with idiopathic inflammatory myopathies (IIM). FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; 6MWD: 6-min walk distance.

**FIGURE 3d**
Monitoring approach for patients with interstitial lung disease (ILD) associated with Sjögren disease (SjD) and mixed connective tissue disease (MCTD). CTD: connective tissue disease; FVC: forced vital capacity; FEV₁: forced expiratory volume in 1 s; D_LCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; 6MWD: 6-min walk distance.

**FIGURE 4a**
Treatment algorithms for patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD). HRCT: high resolution computed tomography; MMF: mycophenolate mofetil.

**FIGURE 4b**
Treatment algorithms for patients with rheumatoid arthritis (RA)-associated interstitial lung disease (ILD). UIP: usual interstitial pneumonia; JAK: Janus kinase.

**FIGURE 4c**
Treatment algorithms for patients with interstitial lung disease (ILD) associated with idiopathic inflammatory myopathies (IIM). CNI: calcineurin inhibitor; IVIG: intravenous immunoglobulins; JAK: Janus kinase.

**FIGURE 4d**
Treatment algorithms for other connective tissue disease (CTD)-associated interstitial lung disease (ILD) patients, such as Sjögren disease-, mixed connective tissue disease- or systemic lupus erythematosus-associated ILD.

See this image and copyright information in PMC

Comment in

ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease: a multidisciplinary, evidence-based approach to best management.
Del Galdo F, Thornton LE, George PM. Del Galdo F, et al. Eur Respir J. 2026 Jan 22;67(1):2501973. doi: 10.1183/13993003.01973-2025. Print 2026 Jan. Eur Respir J. 2026. PMID: 41571325 No abstract available.

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

Affiliations

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

Authors

Affiliations

Abstract

Conflict of interest statement

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Comment in

References

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Medical