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18% TO 32% OF PATIENTS
WITH ILDs ARE AT RISK FOR
DEVELOPING A PROGRESSIVE
FIBROSING PHENOTYPE1*

While all patients with IPF have progressive pulmonary fibrosis, there exists a variety of other ILDs that may also develop a
progressive fibrosing ILD.2,3

PATIENTS WITH PROGRESSIVE FIBROSING ILD FOLLOW A SIMILAR
DISEASE COURSE AND PROGNOSIS AS THAT OBSERVED WITH IPF2

Progression of pulmonary fibrosis

Progression of pulmonary fibrosis

Worsening respiratory symptoms

Worsening respiratory symptoms

Decline of pulmonary function

Decline of pulmonary function

In progressive fibrosing ILD, pulmonary fibrosis continues to develop independent
of the originating disease or trigger, along common pathogenic pathways2-5

*Data based on online surveys with 486 physicians.1

The burden of progressive fibrosing ILD can substantially reduce
a patient's health-related quality of life2

In addition to the functional impact, patients with progressive fibrosing ILD may suffer emotional distress, including feelings of depression, loss, and powerlessness.1,6

ILDs THAT CAN DEVELOP INTO
PROGRESSIVE FIBROSING ILD INCLUDE:1,3

Male patient with an ILD that can develop into progressive fibrosing
  • Idiopathic nonspecific interstitial pneumonia
  • Unclassifiable idiopathic interstitial pneumonia
  • Hypersensitivity pneumonitis
  • Sarcoidosis-associated ILD

See the impact of progressive fibrosing ILDs across a wide range of ILDs

Female patient with a CTD-ILD that can develop into progressive fibrosing

Up to 3 in 10 patients with CTD-ILD including:

  • RA-ILD
  • Sjögren’s syndrome
  • Systemic lupus erythematosus
  • Polymyositis and dermatomyositis ILD
  • Mixed CTD-ILD
  • SSc-ILD
  • Other CTD-ILDs

See the impact of progressive fibrosing ILDs across a range of autoimmune diseases

Slowing disease progression is of critical importance in patients with a progressive fibrosing
ILD as the risk of early death is heightened2

PROGRESSIVE FIBROSING ILD PHENOTYPE
 

 

Dr Nazia Chaudhuri explains the progressive fibrosing ILD phenotype

HOW SHOULD YOU MONITOR PATIENTS WITH PROGRESSIVE FIBROSING ILD?

 

See what Professor Toby Maher has to say
 

Refer patients with a progressive fibrosing ILD for pulmonary rehabilitation as soon as
possible to help keep them active and preserve their quality of life6

Find out how you can make a difference.
BENEFITS OF SUPPORTIVE CARE
References

  1. 1.

    Wijsenbeek M, Kreuter M, Fischer A, et al. Non-IPF progressive fibrosing interstitial lung disease (PF-ILD): the patient journey. Am J Respir Crit Care Med. 2018;197:1-2.

  2. 2.

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

  3. 3.

    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.

  4. 4.

    Selman M, King TE Jr, Pardo A. Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy. Ann Intern Med. 2001;134(2):136-151.

  5. 5.

    Bagnato G, Harari S. Cellular interactions in the pathogenesis of interstitial lung diseases. Eur Respir Rev. 2015;24(135):102-114.

  6. 6.

    Morisset J, Dubè B-P, Garvey C, et al The unmet educational needs of patients with interstitial lung disease, Setting the stage for tailored pulmonary rehabilitation. Ann Am Thorac Soc.2016;13(7):1026-1033.

  7. 7.

    Maher TM, Wuyts W. Management of Fibrosing Interstitial Lung Diseases. Adv Ther. 2019;https://doi.org/10.1007/s12325-019-00992-9.