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Clinical guidelines

Guiding ILD management in systemic autoimmune rheumatic diseases

The first guidelines for the screening, monitoring and treatment of interstitial lung disease (ILD) in patients with systemic autoimmune rheumatic diseases (SARDs) are now available after a major multidisciplinary effort by the ACR and the American College of Chest Physicians. These guidelines demonstrate that multidisciplinary collaborations can improve SARD-ILD management.

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References

  1. Travis, W. D. et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am. J. Respir. Crit. Care Med. 188, 733–748 (2013).

    Article  PubMed  PubMed Central  Google Scholar 

  2. Luppi, F. et al. Lung complications of Sjogren syndrome. Eur. Respir. Rev. 29, 200021 (2020).

    Article  PubMed  PubMed Central  Google Scholar 

  3. Johnson, S. R. et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline for the screening and monitoring of interstitial lung disease in people with systemic autoimmune rheumatic diseases. Arthritis Rheumatol. 76, 1201–1213 (2024).

    Article  PubMed  Google Scholar 

  4. Johnson, S. R. et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic diseases. Arthritis Rheumatol. 76, 1182–1200 (2024).

    Article  PubMed  Google Scholar 

  5. Flaherty, K. R. et al. Nintedanib in progressive fibrosing interstitial lung diseases. N. Engl. J. Med. 381, 1718–1727 (2019).

    Article  CAS  PubMed  Google Scholar 

  6. Levi, Y. et al. Rheumatological assessment is important for interstitial lung disease diagnosis. J. Rheumatol. 45, 1509–1514 (2018).

    Article  PubMed  Google Scholar 

  7. Fischer, A. & Du Bois, R. M. Interstitial lung disease in connective tissue disorders. Lancet 380, 689–698 (2012).

    Article  PubMed  Google Scholar 

  8. Du Bois, R. M. et al. 6-minute walk distance is an independent predictor of mortality in patients with idiopathic pulmonary fibrosis. Eur. Respir. J. 43, 1421–1429 (2014).

    Article  PubMed  Google Scholar 

  9. Matteson, E. L. et al. Nintedanib in patients with autoimmune disease-related progressive fibrosing interstitial lung diseases: subgroup analysis of the INBUILD trial. Arthritis Rheumatol. 74, 1039–1047 (2022).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Solomon, J. J. et al. Safety, tolerability, and efficacy of pirfenidone in patients with rheumatoid arthritis-associated interstitial lung disease: a randomised, double-blind, placebo-controlled, phase 2 study. Lancet Respir. Med. 11, 87–96 (2023).

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

The work of F.L. is partially supported by a grant from the Italian MUR Dipartimenti di Eccellenza 2023–2027 (l. 232/2016, art. 1, commi 314–337).

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Correspondence to Fabrizio Luppi.

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Luppi, F., Sebastiani, M. Guiding ILD management in systemic autoimmune rheumatic diseases. Nat Rev Rheumatol (2024). https://doi.org/10.1038/s41584-024-01160-2

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