EULAR and ERS avoid the ACR road, break down advice by CTD type.
European experts have released new international guidelines on screening, monitoring and treating patients with connective tissue disease-associated interstitial lung disease (CTD-ILD).
Developed jointly by the European Respiratory Society (ERS) and the European Alliance of Associations for Rheumatology (EULAR), and endorsed by ERN-LUNG, the guidelines bring together the most comprehensive evidence-based recommendations to date for managing ILD in systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myopathies, Sjögren’s disease, systemic lupus erythematosus and mixed connective tissue disease.
The guidelines have been published this month in the Annals of the Rheumatic Diseases.
“Interstitial lung disease (ILD) is one of the most frequent manifestations in connective tissue diseases (CTDs) and is associated with high morbidity and mortality,” the authors wrote.
“International collaborations by pulmonology and rheumatology societies including patient research partners are important to develop guidelines with evidence-based approaches for screening, diagnosis, monitoring and treatment of ILD in CTDs for optimised management in clinical practice.”
The task force committee concluded with recommendations for 25 PICO and 28 narrative questions, regarding ILD in the context of the systemic sclerosis, RA, idiopathic inflammatory myopathies, Sjögren’s disease, SLE and MCTD.
Screening, diagnostic, monitoring and treatment algorithms were developed based on the recommendations and usual clinical practice.
The task force recommended high-resolution CT screening at diagnosis for all patients with systemic sclerosis, mixed connective tissue disease and myositis patients with risk factors, while suggesting selective screening for rheumatoid arthritis and Sjögren’s disease in those at risk.
Routine lung biopsy is discouraged except where alternative diagnoses are strongly suspected.
At diagnosis, all patients should undergo risk assessment with pulmonary function tests, HRCT, six-minute walk test and patient-reported outcome measures.
High-risk patients should be monitored closely with PFTs every three to six months and HRCT after 12 months, while lower-risk patients may be reviewed at longer intervals.
Treatment advice is tailored to disease subtype but emphasises early and sometimes aggressive intervention for patients with progressive or severe ILD.
Mycophenolate mofetil, rituximab, cyclophosphamide and tocilizumab are recommended for systemic sclerosis-ILD, while nintedanib, alone or in combination with MMF, is suggested for progressive fibrosis.
Rheumatoid arthritis-ILD should be managed with immunosuppression, with pirfenidone an option in patients with a UIP pattern.
Myositis-related ILD should be treated with immunosuppression, often in combination with glucocorticoids, and immunosuppression is also recommended for Sjögren’s disease, SLE- and MCTD-related ILD.
Related
Across all CTDs, patients who develop progressive pulmonary fibrosis may benefit from nintedanib, according to the guidelines.
Non-pharmacological and supportive care remain integral alongside pharmacological therapy.
Despite the breadth of the recommendations, most are conditional due to low or very low certainty of evidence, reflecting the rarity of CTD-ILD and the lack of large-scale trials.
The guideline committee has identified urgent research priorities to address these gaps.
For Australian clinicians, the document reinforces the importance of early ILD detection and structured, multidisciplinary management, with a particular focus on patient-reported outcomes to capture disease burden and guide therapy.
