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

Area of Science:

• Pulmonology and Rheumatology
• Clinical Practice Guidelines
• Evidence-Based Medicine

Background:

• Interstitial lung disease (ILD) is a serious complication of connective tissue diseases (CTDs), leading to significant morbidity and mortality.
• Standardized approaches to screening, diagnosis, treatment, and follow-up of CTD-ILD are crucial for optimizing patient care.

Purpose of the Study:

• To develop evidence-based clinical practice guidelines for the management of ILD in patients with various CTDs.
• To provide standardized recommendations for screening, diagnosis, treatment, and monitoring of CTD-ILD.

Main Methods:

• A joint task force of the European Respiratory Society and European Alliance of Associations for Rheumatology convened.
• Recommendations were formulated based on PICO and narrative questions, using the GRADE methodology and Evidence to Decision framework.
• Expert consensus from pulmonologists, rheumatologists, pathologists, radiologists, methodologists, and patient representatives guided the process.

Main Results:

• The task force generated recommendations for 25 PICO and 28 narrative questions across multiple CTDs, including systemic sclerosis, rheumatoid arthritis (RA), idiopathic inflammatory myopathies, Sjögren disease (SjD), systemic lupus erythematosus (SLE), and mixed connective tissue disease (MCTD).
• Insufficient evidence precluded recommendations for specific aspects of ILD progression risk assessment in MCTD, SjD, and SLE, and for pirfenidone use in non-RA CTD-ILD.
• Screening, diagnostic, monitoring, and treatment algorithms were developed to support clinical practice.

Conclusions:

• The guidelines provide practical, evidence-based recommendations for clinicians managing CTD-ILD.
• Significant gaps in evidence were identified, highlighting the need for further research to refine management strategies.
• The recommendations aim to standardize care and improve outcomes for patients with CTD-associated interstitial lung disease.