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WHAT IS PPF?

PPF is a fatal condition of worsening lung scarring and declining lung function1

PPF can occur in a wide range of interstitial lung diseases (ILDs)2,3

  • HP
  • Autoimmune ILDs
 
    • RA-ILD
 
    • SSc-ILD
 
    • Mixed CTD-ILD
  • Exposure related
  • Sarcoidosis
  • IIPs other than IPF
  • Other ILDs
wht-is-ppf
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CTD, connective tissue disease; HP, hypersensitivity pneumonitis; IIP, idiopathic interstitial pneumonia; IPF, idiopathic pulmonary fibrosis; RA, rheumatoid arthritis; SSc, systemic sclerosis.

URGENCY TO TREAT

The prognosis for PPF is dire, with significant burden on already vulnerable patients2,4,5

Patients with PPF are at risk of:

worsening-of-symptoms-like
Worsening of symptoms like dyspnea, cough, and fatigue2,6,7
morbidity
Morbidity and mortality, as the median survival from symptom onset to death is 5-6.5 years2,8
lung
Permanent loss of lung function even without worsening symptoms2,9,10

PPF has the potential to progress quickly and suddenly, compromising physical and mental well-being2,6 

TREATMENT BURDEN

Patients with PPF are being held back by limitations of previously approved treatments11,12

Urgent action is a key facet in slowing disease progression and improving outcomes, but finding the right treatment can be challenging12

immunosuppressants
Immunosuppressants

Are often cycled through to manage inflammation from underlying conditions, but may not be enough to address fibrosis alone11

antifibrotics
Antifibrotics

Provide modest benefit, can be difficult to tolerate (eg, GI side effects), and are often added too late when fibrosis is already advanced9,11,13

Expert consensus estimates that up to 32% of non-IPF ILD patients progress despite initial management including immunosuppressive therapy14

Patients need an earlier, better-tolerated treatment plan that addresses both inflammation and fibrosis to reduce FVC decline

FVC, forced vital capacity; GI, gastrointestinal.

Discover a revolutionary treatment option for patients with PPF

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References

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

  2. Kolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20(1):57.

  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(150):180076.

  4. Lee H, Kim SY, Park YS, Choi SM, Lee JH, Park J. Prognostic implication of 1-year decline in diffusing capacity in newly diagnosed idiopathic pulmonary fibrosis. Sci Rep. 2024;14(1):8857.

  5. Martinez FJ, Collard HR, Pardo A, et al. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017;3:17074.

  6. Swigris JJ, Brown KK, Abdulgawi R, et al. Patients’ perceptions and patient-reported outcomes in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180075.

  7. Reichmann WM, Yu YF, Macaulay D, Wu EQ, Nathan SD. Change in forced vital capacity and associated subsequent outcomes in patients with newly diagnosed idiopathic pulmonary fibrosis. BMC Pulm Med. 2015;15:167.

  8. Fan JJ, Gu JM, Xiao SY, Jia MY, Han GL. Risk factors for progression of pulmonary fibrosis: a single-centered, retrospective study. Front Med (Lausanne). 2024;11:1335758.

  9. Denis A, Tsiri P, Guiot J, et al. A new era in the treatment of progressive fibrosing interstitial lung diseases. Breathe (Sheff). 2025;21(3):240259.

  10. Castelino FV, Moua T. Detection and management of interstitial lung diseases associated with connective tissue diseases. ACR Open Rheum. 2021;3(5):295-304.

  11. Khor YH, Moor CC, Merkt W, et al. Treating connective tissue disease-associated interstitial lung disease - think outside the box: a perspective. Eur Respir Rev. 2025;34(177):250046.

  12. Wijsenbeek M, Cottin V. Spectrum of fibrotic lung diseases. N Engl J Med. 2020;383(10):958-968.

  13. Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381(18):1718-1727.

  14. Rajan SK, Cottin V, Dhar R, et al. Progressive pulmonary fibrosis: an expert group consensus statement. Eur Respir J. 2023;61(3):2103187.

MLR ID: PC-AE-102895
Expiry Date: 10/05/2028