Patients with Sjögren disease (SjD), mixed connective tissue disease (MCTD), or systemic lupus erythematosus (SLE) who develop interstitial lung disease represent a specific clinical population in whom the underlying systemic autoimmune condition directly shapes treatment decision-making.
This protocol addresses adults with confirmed SjD, MCTD, or SLE in whom ILD has developed. The co-existence of active connective tissue disease and pulmonary involvement requires an integrated approach that accounts for both systemic disease activity and the trajectory of lung impairment.
Immunosuppressive therapy is the cornerstone of management. The choice of agent — and whether single or combined immunosuppression is appropriate — depends on the degree of disease severity, risk of progression, and extent of organ involvement.
The primary therapeutic targets are improvement or stabilisation of FVC % and DLCO % — pulmonary function parameters that reflect disease trajectory and guide assessment of treatment response.
DOI: 10.1183/13993003.02533-2024
We suggest using immunosuppressive treatment in patients with SjD, MCTD and SLE-ILD (conditional recommendation, very low certainty of evidence; no studies included).
We suggest treating patients with any CTD-ILD with a combination of immunosuppressants or, in the presence of progressive pulmonary fibrosis, with a combination of an immunosuppressant and nintedanib (conditional recommendation, very low certainty of evidence; no studies included).
We suggest using immunosuppressive therapy for other CTD-ILD based on indirect evidence indicating potential improvement or stabilisation in FVC % and DLCO %, with an acceptable safety profile.
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