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Update on treatment of fibrotic ILD

Presented by
Dr Elisabeth Bendstrup, Aarhus University Hospital, Denmark
NLC 2022
The definition of progressive fibrotic-interstitial lung disease (PF-ILD) has been replaced by the more adequate definition of progressive pulmonary fibrosis (PPF). Antifibrotic therapy has shown to benefit patients with idiopathic pulmonary fibrosis (IPF)/PPF; and with an increasing number of new treatment modalities currently in the pipeline, outcomes for patients will – hopefully – continue to improve in the future.

The ATS/ERS/JRS/ALAT Clinical Practice Guidelines have proposed a new definition of PPF which requires at least two of three criteria within the past year without an alternative explanation in a patient with an ILD other than IPF (see Table) [1].

Table: New definition of PPF according to ATS/ERS/JRS/ALAT Clinical Practice Guidelines [1]
In a patient with ILD of known or unknown aetiology other than IPF who has radiological evidence of pulmonary fibrosis, PPF is defined as at least two of the following three criteria occurring within the past year with no alternative explanation*:

1. Worsening of respiratory symptoms

2. Physiological evidence of disease progression (either of the following):

a) Absolute decline in forced vital capacity >5% predicted within 1 year of follow-up

b) Absolute decline in diffusing capacity for carbon monoxide (corrected for Hb) >10% predicted within 1 year of follow-up

3. Radiological evidence of disease progression (one or more of the following):

a) Increased extent or severity of traction bronchiectasis and bronchiolectasis

b) New ground-glass opacity with traction bronchiectasis

c) New fine reticulation

d) Increased extent or increased coarseness of reticular abnormality

e) New or increased honeycombing

f) Increased lobar volume loss

*Although it is critical to exclude alternative factors of worsening in patients with suspected RA-ILD progression, this particularly applies to patients with worsening of respiratory symptoms and/or decline in diffusing capacity for carbon monoxide given the lower specificity of these features for PPF compared with forced vital capacity and chest computed tomography.

“This new definition ticks all the boxes,” according to Dr Elisabeth Bendstrup (Aarhus University Hospital, Denmark). “First of all, this definition takes into account that disease progression is the result of PPF beyond the interstitial space in lung parenchyma. Secondly, disease progression causes a clinical course which is very similar to IPF. Moreover, the definition of PPF is simple and compatible with the broadly used term of pulmonary fibrosis among both clinicians and patients [1].”

“Management of ILD has various aspects,” Dr Bendstrup outlined. “It ranges from an ‘expectative approach’ to anti-fibrotic or immunomodulatory treatment and supportive or palliative care, as well as the treatment of comorbidities and lung transplantation.” Increasing insights into the underlying mechanisms of IPF have driven the development of more effective treatment modalities. However, although nintedanib and pirfenidone significantly reduce lung function decline over time, IPF still cannot be cured, underscoring the ongoing unmet need for effective treatment of this disease [2]. “Current guidelines offer a conditional recommendation for the treatment of IPF with both drugs, as well as a conditional recommendation for the treatment of PPF with nintedanib in patients who have failed standard management for fibrotic ILD. Furthermore, the guidelines also advocate further research on effectiveness of pirfenidone in PPF [1].” With regard to new pharmacological treatment modalities, Dr Bendstrup mentioned several classes of candidate drugs currently under investigation aiming at different molecular targets in IPF and/or fibrotic ILD, e.g. endothelin receptor antagonists, angiogenesis inhibitors, cytokine/kinase inhibitors, antioxidants, vasodilators, and anti-inflammatory/immunosuppressive agents [3].

  1. Raghu G, et al. Adv Ther. 2022;205(9):e18–e47.
  2. Somogyi V, et al. Eur Respir Rev. 2019 Sep 4;28(153):190021.
  3. Bendstrup E. Treatment of fibrotic ILD; where are we now and where are we going? Nordic Lung Congress 2022, 01–03 Jun, Copenhagen, Denmark.


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