Monitoring for ILD progression

Worsening of symptoms or decline in PFT scores can mean that a patient’s pulmonary fibrosis is progressing1

Three patients holding lung scan results in their hands.

A proportion of patients with ILDs may develop a progressive fibrosing phenotype.2–5

Once alternative explanations, such as respiratory tract infection, have been excluded and treated, patients with ILDs meeting the following criteria within 24 months despite treatment in clinical practice can be considered to have progressive fibrosis:6

  • Relative decline of ≥10% in FVC

  • Relative decline of ≥5–<10% in FVC and relative decline of ≥15% in DLco

  • Relative decline of ≥5–<10% in FVC and worsening of respiratory symptoms

  • Relative decline of ≥5–<10% in FVC and increased extent of fibrosis on HRCT

  • Worsening of respiratory symptoms and increased extent of fibrosis on HRCT

 

PATIENTS WITH PROGRESSIVE FIBROSING ILD FACE A DISMAL PROGNOSIS2

Progressive fibrosing ILD has a significant impact on patients, including early mortality:2–5,7

Arrow pointing down in the graph representing a poor survival following an acute exacerbation of IPF.

ILDs can cause progressive deterioration in lung function, respiratory symptoms and quality of life3,4,8–11

Icon of lungs affected by the extent of fibrosis.

If ILD cannot be stabilized within 6–12 months, patients with cHP and NSIP will have ongoing lung function decline and increased mortality6

Progressive fibrosing ILD is characterized by progression of pulmonary fibrosis, worsening of symptoms, decline in lung function and deterioration in health-related quality of life4

Characteristics and potential impacts of the progressive fibrosing phenotype

Find out more about the impacts of the progressive fibrosing phenotype

 

EARLY AND REGULAR MONITORING OF PULMONARY FIBROSIS IN PATIENTS IS IMPORTANT TO DETECT THE PROGRESSIVE FIBROSING PHENOTYPE, AS SOON AS POSSIBLE11,12

Time is of the essence when progressive fibrosing ILD is suspected:3

  • Delayed detection of progressive pulmonary fibrosis in ILDs can be deadly: patients have an estimated survival time of 2.5–4 years after detection of progressive fibrosis2–3†

  • Early detection of patients with progressive fibrosing ILD could inform management decisions and patient counseling2

 

CAN YOU SHORTEN THE TIME BETWEEN ILD DIAGNOSIS AND DETECTION OF THE PROGRESSIVE FIBROSING PHENOTYPE IN YOUR PATIENTS?

In an online physician survey, it was estimated that it takes 9–12 months for the progressive fibrosing phenotype to be detected after it develops2†

Estimated time periods between initial ILD symptoms and detection of the progressive fibrosing phenotype in patients with different ILDs2†

Average waiting time for ILD diagnosis


Current Medical Research and Opinion, 2019, reprinted by permission of the publisher Informa UK Limited trading as Taylor & Francis Ltd.
Patient journey in non-IPF progressive fibrosing ILDs as estimated by an online physician survey.2†
†  Estimates of patients with non-IPF ILD that develop progressive fibrosis based on an online survey of total 486 physicians (243 pulmonologists, 203 rheumatologists and 40 internists) from the United States, Japan, France, Germany, Italy, Spain and the United Kingdom.2

Multidisciplinary evaluation is the gold standard in the evaluation of ILD severity13

A multidisciplinary team including a pulmonologist, radiologist and histopathologist should be involved in ILD diagnosis

Diagnosis and monitoring of ILDs that may present a progressive fibrosing phenotype3

Diagnosis and monitoring algorithm of progressive fibrosing ILDs

Adapted from: Cottin V, Hirani N, Hotchkin D, et al. Presentation, diagnosis and clionical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180076.

MONITORING FOR THE PROGRESSIVE FIBROSING PHENOTYPE WITH PFTs, HRCT AND SYMPTOM EVALUATION

Icon of person coughing representing worsening respiratory symptoms.

Worsening respiratory symptoms

HRCT scan icon.

HRCT

Spirometer icon.

PFT

Monitoring ILD progression using PFTs: 

  • Deterioration in PFTs should trigger urgency for further investigation — because pulmonary fibrosis is unpredictable1,14

  • A decline in DLCO is often considered a sign of progressive ILD, particularly when accompanied by a decline in FVC or increased radiological evidence of fibrosis on HRCT over time6

 

Confirmation of ILD progression using HRCT: 

  • For patients at risk of developing a progressive fibrosing phenotype, HRCT should be evaluated upon worsening of either PFT scores, or respiratory symptoms3,15–17

 

Other considerations when monitoring for ILD progression

Icon of down graph symbol illustrating seriousness in patients with chronic fibrotic HP.

Acute exacerbation of ILD can be considered an indicator of progressive fibrosis, as lung function irreversibly declines as a result6

Icon representing improvement after pulmonary rehabilitation in patients with ILD

Serial measurement of 6MWD can be helpful in identifying progressive fibrosing ILD, as decline in the 6MWD can provide supportive evidence for progressive fibrosis6

CONSIDERATIONS FOR MONITORING OF SPECIFIC ILDs 

SYSTEMIC SCLEROSIS-ASSOCIATED INTERSTITIAL LUNG DISEASE (SSc-ILD)

Due to the variable and unpredictable nature of pulmonary fibrosis in SSc, vigilant ongoing monitoring should be conducted, with HRCT repeated upon worsening of  PFT scores, or respiratory symptoms:11,18–21

  • All patients with SSc-ILD need to be followed up every 3–6 months with PFTs, and repeated HRCT as guided by clinical decision19,20

 

Monitoring algorithm of SSc-ILD19

Algorithm for SSc-ILD screening / diagnosis recommending primary tool HRCT

Algorithm provides brief summary of evidence-based consensus statements (including supplemental Delphi process) for identification and management of SSc-ILD for use in clinical practice. Data from patients with SSc-ILD should be extrapolated to other CTDs with caution.
This article was published in The Lancet Rheumatology, 2(2), Hoffmann-Vold et al, The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements, E71-E83, Copyright Elsevier 2022.

 

 

RHEUMATOID ARTHRITIS-ASSOCIATED INTERSTITIAL LUNG DISEASE (RA-ILD)

Follow-up is mandatory in patients with asymptomatic RA-ILD to identify progressive disease:22

  • Monitoring for progression of RA-ILD using PFTs is recommended every 3–6 months23

What management options should you consider for your patients with fibrotic ILDs?

Footnotes
  • 6MWD, six-minute walking distance; BAL, bronchoalveolar lavage; cHP, chronic hypersensitivity pneumonitis; CTD-ILD, connective tissue disease-associated interstitial lung disease; DLCO, diffusing capacity of the lung for carbon; FVC, forced vital capacity; HP, hypersensitivity pneumonitis; HRCT, high-resolution computed tomography; HRQoL, health-related quality of life; IIP, idiopathic interstitial pneumonia; ILD, interstitial lung disease; iNSIP, idiopathic non-specific interstitial pneumonia; MDT, multidisciplinary team; NSIP, non-specific interstitial pneumonia; PFT, pulmonary function test; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease.

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  2. 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(11):2015–2024.

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  9. Lamas D, Kawut SM, Bagiella E, et al. Delayed Access and Survival in Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2011;184(7):842–847.

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