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New guidelines for IPF and PPF

Presented by
Dr Elisabeth Renzoni, Royal Brompton Hospital, UK
ATS 2022
A team of multidisciplinary experts reviewed and updated the clinical practice guidelines for idiopathic pulmonary fibrosis (IPF), and presented them at ATS 2022. Furthermore, the committee of experts defined progressive pulmonary fibrosis (PPF) in patients with non-IPF interstitial lung diseases (ILDs) and made recommendations with regard to the treatment of this condition. These recommendations were recently also published.

Dr Elisabeth Renzoni (Royal Brompton Hospital, UK) presented the new guidelines at ATS 2022 [1]. She started by explaining that IPF can be identified through radiological and histologic characteristics of usual interstitial pneumonia (UIP). A diagnosis of UIP via biopsy is based on a set of histopathological features. Although transbronchial lung cryobiopsy (TBLC) is more likely to detect a probable UIP pattern than a definite UIP pattern compared with surgical lung biopsy (SLB), a novel systematic review [3] has been published since the development of the IPF guidelines of 2018 [4], demonstrating that TBLC may be a valid alternative to SLB in experienced centres. Therefore, the committee made the conditional recommendation that TBLC may be an alternative to SLB when making a histopathological diagnosis in patients with ILD.

The committee did not make a recommendation for the use of an additional genomic classifier test in patients with ILD who are being diagnosed for UIP through transbronchial forceps biopsy, due to a lack of consensus between the members. Although the systematic review [5] that was analysed to make an informed decision on this topic did not lead to a recommendation, the experts agreed that the use of genomic classifier testing should be revised if new studies are being published.

Furthermore, 2 treatment-related recommendations were made. Firstly, a conditional recommendation was made to not use antacid medication in patients with IPF if the goal is to improve respiratory outcomes. A recently published systematic review [6] did not reveal definitive benefits of antacid medication for the purpose of treating respiratory issues in patients with IPF. However, antacid medication may still be appropriate in patients with IPF and gastro-oesophageal reflux disease (GERD) for treating GERD-related outcomes.

Additionally, it was suggested by the committee (conditional recommendation) that patients with IPF should not be referred to anti-reflux surgery with the goal of improving respiratory outcomes. The systematic review [6] analysing this matter did not demonstrate a significant respiratory benefit for this type of surgery in patients with IPF, but did show that surgical complications occurred in approximately 15% of the patients. Nonetheless, anti-reflux surgery may still be appropriate in patients with IPF to treat GERD-related outcomes

In patients with ILD, other than IPF, and with radiological evidence of pulmonary fibrosis, PPF was defined by the committee as the occurrence of at least 2 of the following 3 features, within the last 12 months, if no alternative explanation was present: worsening of respiratory symptoms, physiological evidence of disease progression, and radiological evidence of disease progression.

In addition, the committee made a conditional recommendation to use nintedanib for the treatment of PPF in patients with fibrotic ILD (other than IPF) who failed on standard management, but emphasised that research is needed to establish the performance of nintedanib in specific ILDs that display PPF. This recommendation was based on a systematic review that demonstrated significant efficacy of nintedanib in patients with PPF, measured as the annual decline of forced vital capacity (FVC), without persistent adverse events if the therapy was discontinued [7].

Finally, the experts recommended that research needs to be conducted to assess the efficacy, effectiveness, and safety of pirfenidone in patients with ILD (non-IPF) who display PPF. Although the committee agreed that pirfenidone is a promising therapy for PPF, the systematic review that was studied by the group did not provide sufficient evidence to make a recommendation.

  1. Renzoni E, et al. Recent ILD Guidelines. Session PG10, ATS International Conference 2022, San Francisco, CA, USA, 13–18 May.
  2. Raghu G, et al. Am J Respir Crit Care Med. 2022;205(9):e18–e47.
  3. Kheir F, et al. Ann Am Thorac Soc. 2022; May 2. doi:10.1513/AnnalsATS.202102-198OC. Online ahead of print.
  4. Raghu G, et al. Am J Respir Crit Care Med 2018;198:e44–e68.
  5. Kheir F, et al. Ann Am Thorac Soc. 2022;19(5):827–832.
  6. Khor YH, et al. Ann Am Thorac Soc. 2022;19(5):833–844.
  7. Ghazipura MHM, et al. Ann Am Thorac Soc. 2022; May 2. doi:10.1513/AnnalsATS.202103-343OC. Online ahead of print.



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