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Background <p>Systemic rheumatic diseases with interstitial lung disease (SARD-ILD) are often associated with progressive pulmonary fibrosis (PPF), characterised by increasing respiratory symptoms, progressive loss of lung function, and an unfavourable prognosis. Currently available therapies, including glucocorticoids, immunosuppressants and antifibrotic agents, often have limited efficacy depending on the underlying SARD-ILD, while adverse events limit their use. Therefore, there is a&#xa0;substantial unmet need for effective and well-tolerated treatment options.</p> Objectives <p>This review summarises current developments and emerging therapeutic approaches in SARD-ILD and PPF, highlighting their potential relevance for clinical practice.</p> Materials and methods <p>A&#xa0;systematic PubMed search (publications up to mid-2025) using the terms “SARD-ILD”, “CTD-ILD”, “PPF” and “treatment” identified relevant studies investigating novel therapeutic strategies.</p> Results <p>Substances currently under clinical investigation include nerandomilast, belimumab, admilparant, anifrolumab and inhaled treprostinil. Nerandomilast, a&#xa0;preferential PDE4B inhibitor with antifibrotic and anti-inflammatory properties, demonstrated a&#xa0;reduction in lung function loss and mortality risk with good tolerability in the phase‑3 FIBRONEER-ILD study, potentially representing a&#xa0;turning point in the management of autoimmune-associated ILD.</p> Conclusion <p>Promising results from ongoing clinical studies provide new therapeutic perspectives for SARD-ILD and PPF and may significantly improve prognosis and disease burden in the future.</p>

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Management von SARD-ILD im Wandel

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年3月25日

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  • Torsten Witte,
  • Jonas Schupp

摘要

Background

Systemic rheumatic diseases with interstitial lung disease (SARD-ILD) are often associated with progressive pulmonary fibrosis (PPF), characterised by increasing respiratory symptoms, progressive loss of lung function, and an unfavourable prognosis. Currently available therapies, including glucocorticoids, immunosuppressants and antifibrotic agents, often have limited efficacy depending on the underlying SARD-ILD, while adverse events limit their use. Therefore, there is a substantial unmet need for effective and well-tolerated treatment options.

Objectives

This review summarises current developments and emerging therapeutic approaches in SARD-ILD and PPF, highlighting their potential relevance for clinical practice.

Materials and methods

A systematic PubMed search (publications up to mid-2025) using the terms “SARD-ILD”, “CTD-ILD”, “PPF” and “treatment” identified relevant studies investigating novel therapeutic strategies.

Results

Substances currently under clinical investigation include nerandomilast, belimumab, admilparant, anifrolumab and inhaled treprostinil. Nerandomilast, a preferential PDE4B inhibitor with antifibrotic and anti-inflammatory properties, demonstrated a reduction in lung function loss and mortality risk with good tolerability in the phase‑3 FIBRONEER-ILD study, potentially representing a turning point in the management of autoimmune-associated ILD.

Conclusion

Promising results from ongoing clinical studies provide new therapeutic perspectives for SARD-ILD and PPF and may significantly improve prognosis and disease burden in the future.