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Progressive Pulmonary Fibrosis: Current Status in Terminology and Future Directions

Yasuhiro Kondoh1,2, Yoshikazu Inoue3,4

  • 1Department of Respiratory Medicine and Allergology, Aichi Medical University, 1-1 Yazakokarimata, Nagakute, Aichi, 480-1195, Japan. yshr.kondoh@gmail.com.

Advances in Therapy
|May 19, 2025
PubMed

Insights

New clinical guidelines propose "progressive pulmonary fibrosis" (PPF) for non-IPF fibrosing interstitial lung diseases (ILDs). This commentary clarifies PPF and contrasts it with the widely used "progressive fibrosing interstitial lung disease" (PF-ILD) term.

Area of Science:

  • Pulmonology
  • Rheumatology
  • Interstitial Lung Diseases

Background:

  • 2022 guidelines introduced "progressive pulmonary fibrosis" (PPF) for non-IPF fibrosing interstitial lung diseases (ILDs).
  • The term PPF aims to define specific criteria for these patients.
  • This new terminology has created confusion due to the established use of "progressive fibrosing interstitial lung disease" (PF-ILD).

Keywords:
ATS/ERS/JRS/ALAT clinical practice guidelinesClinical practice guidelinesINBUILDProgressive fibrosing interstitial lung diseaseProgressive pulmonary fibrosisTerminology

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