Skip to main content

Progressive pulmonary fibrosis (PPF)

Nurses should be conscious of the signs of progression in their patients with fibrotic ILD1–7

Progressive pulmonary fibrosis (PPF)

Fibrotic ILD that progresses is known as progressive pulmonary fibrosis (PPF) or progressive fibrosing ILD.8,9

IDENTIFYING PROGRESSIVE PULMONARY FIBROSIS

Increasing fibrosis, worsening respiratory symptoms, and decline in pulmonary function are each signs of progression that, alone or in combination, can indicate Progressive Pulmonary Fibrosis (PPF).2,7–9

According to international guidelines, PPF is defined as at least two of the following three criteria occurring within the past year with no alternative explanation:*9

Worsening respiratory symptoms

Worsening respiratory symptoms
Physiological (e.g. PFT) evidence of pulmonary function decline

Physiological (e.g. PFT) evidence of pulmonary function decline
Radiological (e.g. HRCT) evidence of pulmonary fibrosis progression

Radiological (e.g. HRCT) evidence of pulmonary fibrosis progression

The ATS/ERS/JRS/ALAT clinical practice guideline gives a consensus-based definition of PPF.9

You have an important role within the multidisciplinary team (MDT) in coordinating investigations and referrals, and liaising with the MDT, when PPF is suspected5,10

DESPITE STANDARD OF CARE, ILD MAY BECOME PROGRESSIVE, DESCRIBED AS PPF1,8,9,11–14

All patients with IPF are expected to develop progressive disease.8,15 In addition to IPF, disease progression occurs in range of other ILDs.1,8,9,12–14

Patients should be made aware that the disease course of ILD can be unpredictable, and that it can be difficult to predict disease progression.1,8,12

An individual patient’s disease course can be unpredictable and survival variable, dependent on the ILD diagnosis and disease severity.1,8,12 The signs and symptoms of progressive disease may develop slowly over time, or rapidly as an acute exacerbation of their ILD.12,14,16

Patients with a range of ILDs may develop progressive pulmonary fibrosis9*

Patients with a range of ILDs may develop progressive pulmonary fibrosis9*

 

*The coral shaded area represents the estimated proportion of patients with various types of ILD who manifest progressive pulmonary fibrosis.

AFOP, acute fibrinous and organizing pneumonia; AIP, acute interstitial pneumonia; COP, cryptogenic organizing pneumonia; HP, hypersensitivity pneumonitis; iDIP, idiopathic desquamative interstitial pneumonia; IIP, idiopathic interstitial pneumonia; ILD, interstitial lung disease; iLIP, idiopathic lymphoid interstitial pneumonia; iNSIP, idiopathic nonspecific interstitial pneumonia; IPF, idiopathic pulmonary fibrosis; iPPFE, idiopathic pleuroparenchymal fibroelastosis; LAM, lymphangioleiomyomatosis; LCH, Langerhans cell histiocytosis; MCTD, mixed connective tissue disease; PAP, pulmonary alveolar proteinosis; RA, rheumatoid arthritis; RBILD, respiratory bronchiolitis interstitial lung disease; SLE, systemic lupus erythematosus; SSc, systemic sclerosis.

Reprinted from Annals of the American Thoracic Society, 205, 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. e18–e47, ©2023.

PROGRESSIVE FIBROSING ILDs ARE ASSOCIATED WITH HIGH MORTALITY12,17

Patients with progressive fibrosing ILD may experience irreversible lung function decline, reduced health-related quality of life, and early mortality.11–13,18

Untreated patients with IPF have a median survival of 3–5 years after diagnosis.12,19 Patients with progressive fibrosing ILDs have a similar disease course to untreated patients with IPF, with an estimated survival time of 4–5 years after diagnosis.14

ILD is the leading cause of mortality in patients with SSc-ILD, being responsible for 16.8–35% of deaths.20,21

Patients with progressive loss of lung function due to fibrotic ILD have a poor prognosis22–33

Patients with progressive loss of lung function due to fibrotic ILD have a poor prognosis22–33

Patients may have questions about disease progression;34 you have an important role in providing education and support to patients and caregivers on difficult subjects, including prognosis5,10

SYMPTOM MONITORING IN PATIENTS WITH ILD

Patients should be made aware that regularly monitoring of their symptoms can help discussions with their doctor about whether their pulmonary fibrosis is worsening.7,8,35

Changes in the symptoms your patients experience, including dry cough, dyspnea, fatigue, and weight loss may indicate that their ILD has become progressive.1,7,12

Patients should be aware it is important to monitor their symptoms and discuss changes with their doctor;1,5,7,35 as symptoms may develop slowly over time,16 it could be useful for them to keep a diary of their symptoms.

Help your patients track their symptoms with a Symptom tracker

Download tracker
symptom tracker

Ensure your patients understand that they should monitor their symptoms and speak to their doctor if they notice any changes5,7,8,35

REGULAR MEDICAL APPOINTMENTS TO MONITOR FOR SIGNS OF PPF

Patients should be made aware that regular check-ups with their doctor are important to detect worsening of their disease as soon as possible.7,8,36,37

Pulmonary fibrosis is unpredictable and regular doctor visits can enable detection of progressive disease; patients should expect further testing including lung imaging, lung function tests, as well as monitoring of their symptoms.1,8,9,35–37

Early detection of PPF with regular tests can help doctors adjust treatment, recommend lifestyle changes and provide additional support.1,2,7,38

Patients with ILDs will be monitored for signs of disease progression8,9,35

Worsening respiratory symptoms

Clinical assessment
PFTs (FVC and DLco)

PFTs (FVC and DLco)
HRCT

HRCT

Remind your patients that it is important to attend their medical appointments, as regular tests can help their doctor provide timely support and adjustment to treatment if needed1,2,7,38,39

What other resources can help you support your patients in their journey with fibrotic ILD?

Footnotes
  • ALAT, Latin American Thoracic Society; ATS, American Thoracic Society; DLco, diffusing capacity of the lung for carbon monoxide; ERS, European Respiratory Society; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; JRS, Japanese Respiratory Society; MDT, multidisciplinary team; PFT, pulmonary function test; PPF, progressive pulmonary fibrosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease.

  1. 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.
  2. George PM, Spagnolo P, Kreuter M, et al. Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities. Lancet Respir Med. 2020;8:925–934.
  3. McLean AEB, Webster SE, Fry M, et al. Priorities and expectations of patients attending a multidisciplinary interstitial lung disease clinic. Respirology. 2021;26:80–86.
  4. Molina-Molina M, Aburto M, Acosta O, et al. Importance of early diagnosis and treatment in idiopathic pulmonary fibrosis. Exp Rev Resp Med. 2018;12(7):537–539.
  5. Russell AM, Olive S, Lines S, et al. Contemporary challenges for specialist nursing in interstitial lung disease. Breathe. 2018;14:36–41.
  6. Volkmann ER, Tashkin DP. Treatment of systemic sclerosis-related interstitial lung disease: A review of existing and emerging therapies. Ann Am Thorac Soc. 2016;13:2045–2056.
  7. Wuyts WA, Wijsenbeek M, Bondue B, et al. Idiopathic pulmonary fibrosis: Best practice in monitoring and managing a relentless fibrotic disease. Respiration. 2020;99:73–82.
  8. Cottin V, Wollin L, Fischer A, et al. Fibrosing interstitial lung diseases: knowns and unknowns. Eur Respir Rev. 2019;28:180100.
  9. 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.
  10. Grewal JS, Ryerson CJ. A closer look at the multidisciplinary interstitial lung disease clinic: Who, what and how. Respirology. 2021;26:12–13.
  11. Flaherty KR, Wells AU, Cottin V et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381:1718–1727.
  12. Kolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20:57.
  13. Maher TM, Wuyts W. Management of fibrosing interstitial lung diseases. Adv Ther. 2019;36:1518–1531.
  14. Wijsenbeek M, Kreuter M, Olson A et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin. 2019;35:2015–2024.
  15. Olson A, Hartmann N, Patnaik P, et al. Estimation of the prevalence of progressive fibrosing interstitial lung diseases: systematic literature review and data from a physician survey. Adv Ther. 2021;38:854–867.
  16. Cosgrove GP, Bianchi P, Danese S, Lederer DJ. Barriers to timely diagnosis of interstitial lung disease in the real world: the INTENSITY survey. BMC Pulm Med. 2018;18:9.
  17. Wijsenbeek M, Cottin V. Spectrum of fibrotic lung diseases. N Engl J Med. 2020;383:958–968.
  18. 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:e000212.
  19. Martinez FJ, Collard HR, Pardo A, et al. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017;3:17074.
  20. Elhai M, Meune C, Boubaya M, et al. Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis. 2017;76:1897–1905.
  21. Tyndall AJ, Bannert B, Vonk M, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69:1809–1815.
  22. Wells AU, Brown KK, Flaherty KR, et al. 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.
  23. Ryerson CJ, Urbania TH, Richeldi L, et al. Prevalence and prognosis of unclassifiable interstitial lung disease. Eur Respir J. 2013;42:750–757.
  24. Hyldgaard C, Bendstrup E, Wells AU, Hilberg O. Unclassifiable interstitial lung diseases: Clinical characteristics and survival. Respirology. 2017;22(3):494–500.
  25. Wells AU, Cullinan P, Hansell DM, et al. Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis. Am J Respir Crit Med.1994;149(6):1583–1590.
  26. Mapel DW, Hunt WC, Utton R, et al. Coultas DB. Idiopathic pulmonary fibrosis: survival in population based and hospital based cohorts. Thorax. 1998;53(6):469–476.
  27. Hubbard R, Johnston I, Britton J. Survival in patients with cryptogenic fibrosing alveolitis: a population-based cohort study. Chest. 1998;113(2):396–400.
  28. Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008;177(11):1248–1254.
  29. Moore OA, Goh N, Corte T, et al. Extent of disease on high-resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease. Rheumatology (Oxford). 2013;52(1):155–160.
  30. Kim EJ, Elicker BM, Maldonado F, et al. Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J. 2010;35(6):1322–1328.
  31. Bongartz T, Nannini C, Medina-Velasquez YF, et al. Incidence and mortality of interstitial lung disease in rheumatoid arthritis: a population-based study. Arthritis Rheum. 2010;62(6):1583–1591.
  32. Gimenez A, Storrer K, Kuranishi L, et al. Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis. Thorax. 2018;73(4):391–392.
  33. Vourlekis JS, Schwarz MI, Cherniack RM, et al. The effect of pulmonary fibrosis on survival in patients with hypersensitivity pneumonitis. Am J Med. 2004;116(10):662–668.
  34. 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.
  35. Cottin V. Treatment of progressive fibrosing interstitial lung diseases: a milestone in the management of interstitial lung diseases. Eur Respir Rev. 2019;28:190109.
  36. Chowaniec M, Skoczyńska M, Sokolik R, Wiland P. Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management. Reumatologia. 2018;56(4):249–254.
  37. Roofeh D, Jaafar S, Vummidi D, Khanna D. Management of systemic sclerosis-associated interstitial lung disease. Curr Opin Rheumatol. 2019;31:241–249.
  38. Wong AW, Ryerson CJ, Guler SA. Progression of fibrosing interstitial lung disease. Respir Res. 2020;21(1):32.
  39. Barbera T, Davila L, Patel NM. Management and support of patients with fibrosing interstitial lung diseases. Nurse Pract. 2021;46(7):39–44.
  40. O’Connor S, Deaton C, Nolan F, Johnston B. Nursing in an age of multimorbidity. BMC Nursing. 2018;17:49.
  41. 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.

Resources for patients