Progressive pulmonary fibrosis (PPF)
Nurses should be conscious of the signs of progression in their patients with fibrotic ILD1–7
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
Physiological (e.g. PFT) evidence of pulmonary function decline
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
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*
*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.
Adapted from: Raghu G, et al. Am J Respir Crit Care Med. 2022;205:e18–e47.
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
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.
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
PFTs (FVC and DLco)
What other resources can help you support your patients in their journey with fibrotic ILD?
Impact of pulmonary fibrosis
- 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.
- 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.
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- 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.
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- 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.
- Cottin V, Wollin L, Fischer A, et al. Fibrosing interstitial lung diseases: knowns and unknowns. Eur Respir Rev. 2019;28:180100.
- 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.
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- Flaherty KR, Wells AU, Cottin V et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381:1718–1727.
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