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

Area of Science:

• Pulmonology and Rheumatology
• Clinical Practice Guidelines
• Connective Tissue Diseases (CTDs)

Background:

• Interstitial lung disease (ILD) is a serious complication of connective tissue diseases (CTDs).
• Standardized care is crucial for managing CTD-ILD, given its high morbidity and mortality.
• Existing guidelines lacked comprehensive, standardized approaches for CTD-ILD.

Purpose of the Study:

• To develop evidence-based clinical practice guidelines for screening, diagnosis, treatment, and follow-up of CTD-ILD.
• To standardize patient care across various connective tissue diseases associated with ILD.
• To provide practical guidance for clinicians managing patients with CTD-ILD.

Main Methods:

• A joint task force of the European Respiratory Society and European Alliance of Associations for Rheumatology was formed.
• Recommendations were developed using PICO questions and complementary narrative questions, with evidence graded by the GRADE methodology.
• The Evidence to Decision framework guided the formulation of recommendations for CTD-ILD.

Main Results:

• Recommendations were established for 25 PICO and 28 narrative questions covering ILD in systemic sclerosis, RA, myopathies, Sjögren's, SLE, and MCTD.
• Insufficient evidence precluded recommendations for ILD progression risk in MCTD, SjD, SLE, and for pirfenidone in non-RA CTD-ILD.
• Screening, diagnostic, monitoring, and treatment algorithms were created based on the recommendations.

Conclusions:

• The guidelines offer practical, evidence-based recommendations for clinicians managing CTD-ILD.
• Significant gaps in evidence were identified, highlighting the need for further research.
• The recommendations aim to optimize patient care and standardize management of CTD-ILD.