Pulmonary fibrosis is a significant threat across a broad range of interstitial lung diseases (ILDs), and calls for urgent identification and intervention.2–6 Nurses have a key role in supporting patients throughout their journey with fibrotic ILD, as well as facilitating and coordinating multidisciplinary care.1,7,9,10

nurses banner


Symptoms of pulmonary fibrosis

Help your patients stay on top of their ILD symptoms. Make sure you are conscious of the common signs and symptoms of fibrotic ILDs, and help your patients effectively communicate about them with their doctor.

Symptoms of pulmonary fibrosis

Resources for you and your patients

Boost your confidence in supporting patients with fibrotic ILD with a broad range of resources. Take a look at the SPIKES model resources to help you broach difficult subjects with patients17,18 and share downloadable materials with your patients to support them throughout their journey with fibrotic ILD.

Resources for you and your patients


  • ILD, interstitial lung disease; MDT, multidisciplinary team; PPF, progressive pulmonary fibrosis.

  1. Russell AM, Olive S, Lines S, et al. Contemporary challenges for specialist nursing in interstitial lung disease. Breathe. 2018;14:36–41.
  2. 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.
  3. Martinez FJ, Collard HR, Pardo A, et al. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017;3:17074.
  4. Spagnolo P, Distler O, Ryerson CJ, et al. Mechanisms of progressive fibrosis in connective tissue disease (CTD)-associated interstitial lung diseases (ILDs). Ann Rheum Dis. 2021;80:143–150.
  5. Volkmann E, Varga J. Emerging targets of disease-modifying therapy for systemic sclerosis. Nat Rev Rheumatol. 2019;15:208–224.
  6. Spagnolo P, Ryerson CJ, Putman R, et al. Early diagnosis of fibrotic interstitial lung disease: challenges and opportunities. Lancet Respir Med. 2021;9(9):1065–1076.
  7. Denton CP, Laird B, Moros L, Flores JLL. Things left unsaid: important topics that are not discussed between patients with systemic sclerosis, their carers and their healthcare professionals – a discourse analysis. Clin Rheumatol. 2021;40:1399–1407.
  8. Grewal JS, Ryerson CJ. A closer look at the multidisciplinary interstitial lung disease clinic: Who, what and how. Respirology. 2021;26:12–13.
  9. Shaw J, Marshall T, Morris H, et al. Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age. J Thorac Dis. 2017;9:4700–4707.
  10. Barbera T, Davila L, Patel NM. Management and support of patients with fibrosing interstitial lung diseases. Nurse Pract. 2021;46(7):39–44.
  11. Walsh SLF. Multidisciplinary evaluation of interstitial lung diseases: current insights. Eur Respir Rev. 2017;26:170002.
  12. De Sadeleer LJ, Meert C, Yserbyt J, et al. Diagnostic ability of a dynamic multidisciplinary discussion in interstitial lung diseases: A retrospective observational study of 938 cases. Chest. 2018;153(6):1416–1423.
  13. Antoniou KM, Margaritopoulos GA, Tomassetti S, et al. Interstitial lung disease. Eur Respir Rev. 2014;23(131):40–54.
  14. Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic Pulmonary Fibrosis (an update) and progressive pulmonary fibrosis in adults. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2022;205:e18–e47.
  15. Wijsenbeek M, Cottin V. Spectrum of fibrotic lung diseases. N Engl J Med. 2020;383:958–968.
  16. Kolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20:57.
  17. Baile WF, Buckman R, Lenzi R, et al. SPIKES – A six-step protocol for delivering bad news: application to the patient with cancer. Oncologist. 2000;5:302–311.
  18. Wijsenbeek MS, Bonella F, Orsatti L, et al. Communicating with patients with idiopathic pulmonary fibrosis: can we do it better? ERJ Open Res. 2022;7:00422–2021.
  19. Bendstrup E, Wuyts W, Alfaro T, et al. Nintedanib in idiopathic pulmonary fibrosis: Practical management recommendations for potential adverse events. Respiration. 2019;97:173–184.
  20. Rahaghi F, Belperio JA, Fitzgerald J, et al. Delphi consensus recommendations on management of dosing, adverse events, and comorbidities in the treatment of idiopathic pulmonary fibrosis with nintedanib. Clin Med Insights Circ Respir Pulm Med. 2021;15:11795484211006050.
  21. Nantz E, Lui-Seifert H, Skljarevski V. Predictors of premature discontinuation of treatment in multiple disease states. Patient Prefer Adherence. 2009;3:31–43.
  22. Kreuter M, Bendstrup E, Russell A, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968–980.

Resources for patients