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Summary

Summary

Summary

  • A common threat across a wide range of ILDs, pulmonary fibrosis can become a key driver of irreversible harm and early mortality that warrants urgent identification and intervention.1-4
  • For at-risk patients, high resolution CT (HRCT) should be evaluated at the first suspicion of ILD involvement—if possible at baseline diagnosis—and repeated upon worsening of either pulmonary function test (PFT) scores or respiratory symptoms.5,6
  • Demonstrate a healthy suspicion: identifying pulmonary fibrosis in your patients as early as possible may help to improve their burden of disease, slow decline in daily functioning and quality of life, and reduce the risk of early mortality.5,7-9
     
References

  1. Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Respir Res. 2017;4(1):e000212.

  2. Patterson KC, Strek ME. Pulmonary fibrosis in sarcoidosis. Clinical features and outcomes. Ann Am Thorac Soc. 2013;10(4):362-370.

  3. Caban JJ, Yao J, Bagci U, Molurra DJ. Monitoring pulmonary fibrosis by fusing clinical, physiological, and computed tomography features. Conf Proc IEEE Eng Med Biol Soc. 2011;2011:6216-6219.

  4. Wells AU, Brown KK, Flaherty KR, Kolb M, Thannickal VJ; IPF Consensus Working Group. What’s in a name? That which we call IPF, by any other name would act the same. Eur Respir J. 2018;51(5):1800692.

  5. Cottin V, Hirani NA, Hotchkin DL, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27:180076.

  6. Raghu G, Collard HR, Egan JJ, et al; on behalf of the ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement:idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.

  7. Fischer A, Distler J. Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases. Clin Rheumatol. 2019. doi:10.1007/s10067-019-04720-0.

  8. Wijsenbeek M, Kreuter M, Fischer A, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin.2019:1–10. DOI: 10.1080/03007995.2019.1647040.

  9. Richeldi L, Varone F, Bergna M, et al. Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence. Eur Respir Rev. 2018;27(150):180074.