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New Guidelines Call for Increased Interstitial Lung Disease Screening in Autoimmune Patients
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BARCELONA, Spain – In a move set to reshape clinical practice, the European Alliance of Associations for Rheumatology (EULAR) and the European Respiratory Society (ERS) have jointly released new guidelines advocating for systematic screening of interstitial lung disease (ILD) in all patients diagnosed with systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The declaration was made at the EULAR 2025 Annual Meeting.
These complete, disease-specific recommendations aim to standardize the screening, diagnosis, and management of ILD across various connective tissue diseases (CTDs), including SSc, MCTD, rheumatoid arthritis (RA), idiopathic inflammatory myopathies (IIM), and Sjögren’s disease (SjD).The guidelines mark a notable step forward, representing the first collaborative effort between rheumatologists and pulmonologists in Europe to address ILD in this patient population.
Screening Recommendations for Interstitial Lung Disease
the cornerstone of the new guidelines is the strong recommendation for systematic ILD screening using high-resolution computed tomography (HRCT) in all SSc and MCTD patients, irrespective of pre-existing risk factors. This recommendation extends to patients with IIM, excluding those with inclusion body myositis. For individuals with RA,SjD,and other CTDs,the guidelines advise an initial assessment for disease-specific risk factors,followed by HRCT screening for those with identified risks.
Did You Know? The American Thoracic Society estimates that ILD affects approximately 15% of patients with rheumatoid arthritis[[1].
While previous recommendations supported screening in SSc, the inclusion of RA patients with risk factors marks a significant expansion. According to Dr. Bernhard Hellmich, this broadened scope is expected to identify more patients who could benefit from early therapeutic intervention, potentially leading to improved long-term outcomes.
Notably, the guidelines do not specify a screening frequency due to limited evidence. Dr. Anna-Maria Hoffmann-Vold suggests mirroring the diagnostic approach and reassessing for new ILD progression risk factors. The guidelines explicitly advise against substituting HRCT with pulmonary function tests or lung ultrasound for initial screening. However, forced vital capacity and diffusing capacity of the lungs for carbon monoxide can be incorporated in the presence of symptoms or CT abnormalities.
Monitoring ILD Progression After Diagnosis
The guidelines provide specific timeframes for monitoring ILD progression post-diagnosis, tailored to individual diseases and stratified by ILD progression risk. Clinicians are advised to assess risk based on prior lung function tests, HRCT results, the 6-minute walking test, and risk factors such as disease-specific circulating markers, disease activity, and HRCT patterns.
Based on these assessments, patients are categorized as high or low risk. Monitoring frequency varies accordingly. for high-risk patients with any CTD, lung function tests are recommended every 3-6 months during the initial years, followed by every 6-12 months thereafter. Lower-risk patients should undergo lung function tests every 6-12 months initially, and then annually.
Treatment Strategies for Interstitial Lung Disease
the new guidelines offer detailed treatment recommendations, outlining available options for each disease and providing guidance on selecting the most appropriate medication for individual patients. The guidelines strongly recommend tocilizumab for SSc-ILD patients with early diffuse SSc and signs of inflammation. For IIM-ILD patients, immunosuppressive treatment, including glucocorticoids, calcineurin inhibitors, rituximab, mycophenolate, or azathioprine, is recommended.
Other treatment recommendations are conditional, reflecting limited patient populations and a scarcity of randomized controlled trials. Individual treatment algorithms are provided for SSc-ILD, RA-ILD, and IIM-ILD.
Dr. Hellmich believes that these comprehensive guidelines will facilitate earlier treatment initiation, potentially improving patient prognosis. He noted that the inclusion of certain therapies in the EULAR guidelines may encourage their use in clinical practice,even if they are currently considered off-label.
Pro Tip: Staying informed about the latest research and guidelines is crucial for providing optimal care to patients with connective tissue diseases and interstitial lung disease.
The full guidelines are slated for publication next month in the Annals of the Rheumatic Diseases and European Respiratory Journal.
