New clinical practice guidelines for interstitial lung disease associated with connective tissue diseases
EULAR—The European Alliance of Associations for Rheumatology—has worked with the European Respiratory Society (ERS) to develop new clinical practice guidelines for interstitial lung disease (ILD) associated with connective tissue diseases (CTD). Such collaboration between pulmonology and rheumatology societies is key to optimizing patient management and improving outcomes across Europe.
CTD is a diverse group of disorders that includes systemic sclerosis (SSc), idiopathic inflammatory myopathies (IIM), Sjögren disease (SjD), systemic lupus erythematosus (SLE) and mixed CTD (MCTD). Rheumatoid arthritis (RA) was also included in this new work. But despite differences in the underlying diseases, ILD is a frequent manifestation across them—and is associated with high morbidity and mortality.
Clinical practice guidelines would help to standardize screening, diagnosis, treatment, and follow-up for patients with CTD-ILD. To address this gap, EULAR and the ERS formed a task force—including pulmonologists and rheumatologists, plus colleagues from radiology and histopathology and patient representatives. A literature review was performed, and the collected evidence was used to formulate recommendations.
The group answered 5 PICO (Patients, Intervention, Comparison, Outcomes) and 10 narrative questions (NQ), resulting in 50 disease-specific recommendations across screening, diagnosis, monitoring, and treatment for patients with CTD-ILD or RA-ILD.
Two simultaneous publications—in the September 2025 issue of the European Respiratory Journal and Annals of Rheumatic Diseases—also include a series of specific ILD management algorithms for each of the underlying rheumatic diseases, which have been developed based on the recommendations and usual clinical practice.
In short, the clinical practice guidelines recommend screening all patients with SSc, MCTD, and IIM (but not people with inclusion body myositis); and patients with RA and SjD who have risk factors for ILD at the time of their CTD diagnosis. The recommended tool is high-resolution computed tomography or HRCT. Lung biopsy does not play a role in identifying CTD-ILD but might be considered in a multi-disciplinary team discussion if alternative diagnoses are possible.
Furthermore, at the time of ILD diagnosis, all patients should be assessed for disease severity, risk of progression, and functional ability. Clinical risk factors can be used to identify people at high or low risk of progression, as well as lung function tests, HRCT scans, 6-minute walking test, and patient-reported outcome measures—which can capture insights about a person's respiratory symptoms and their impact on daily life.
A comprehensive evaluation for all CTD-ILD patients at each visit is suggested to assess their risk for poor prognosis and disease progression, with high-risk patients monitored more frequently with lung function tests, HRCT, and patient-reported outcome measures. People at lower risk should undergo the same assessments but at extended intervals.
Regarding treatment, the choice of therapy should consider patient-specific risk factors, as well as each person's extrapulmonary organ manifestations, potential side effects, and the risk of progressive or severe ILD. Early and/or aggressive treatment is recommended for those at risk of progressive or severe ILD, especially people with multiple risk factors or specific indicators.
Professor Anna-Maria Hoffmann-Vold—corresponding author on the paper, Head of the Inflammatory and Fibrotic Rheumatic Disease Research Area at Oslo University Hospital, and lead of translational research at University Hospital Zurich/Zurich University, said, "The new ERS/EULAR guidelines for CTD-ILD are important because they offer standardized, evidence-based recommendations for patient management, supporting the identification of high-risk individuals and enhancing outcomes.
"The strong collaboration between EULAR and the ERS has played a crucial role in this development, addressing both the rheumatological and pulmonary aspects of these complex conditions, which ultimately benefits our patients."
The task force recognizes that the new guideline has some limitations. The work builds mostly on evidence of low certainty. This is a common challenge for rare diseases and means that most recommendations in the new work are conditional; this gap also reinforces the importance of the ongoing research agenda.
However, the guideline still provides comprehensive support for clinicians and patients with CTD-ILD—and crucially, many pre-defined management areas are addressed specifically for the underlying rheumatic disease, which is important due to some of the inherent differences between diseases.
More information:
Katerina M. Antoniou et al, ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease, European Respiratory Journal (2025). DOI: 10.1183/13993003.02533-2024
Katerina M. Antoniou et al, ERS/EULAR clinical practice guidelines for connective tissue diseases associated interstitial lung disease, Annals of the Rheumatic Diseases (2025). DOI: 10.1016/j.ard.2025.08.021
Provided by EULAR
