Progressive pulmonary fibrosis definition according to pubmed
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ATS ERS JRS ALAT 2022 progressive pulmonary fibrosis definition criteria
Progressive pulmonary fibrosis (PPF) is defined by the 2022 ATS/ERS/JRS/ALAT guideline as:
In a patient with an interstitial lung disease other than idiopathic pulmonary fibrosis (IPF), PPF is present when there are at least 2 of the following 3 features within the past year, with no alternative explanation:
- Worsening respiratory symptoms
- Physiological progression
- Absolute decline in FVC of ≥5% predicted, or
- Absolute decline in DLCO of ≥10% predicted after correction for hemoglobin
- Radiological progression of pulmonary fibrosis on HRCT, such as increased traction bronchiectasis/bronchiolectasis, new or increased reticulation, new ground-glass opacity with traction bronchiectasis, increased honeycombing, or increased lobar volume loss.
The criteria may occur at any point during that one-year interval. PPF is therefore a progressive phenotype of non-IPF fibrotic ILD, not a single specific disease.
Source: Raghu et al., ATS/ERS/JRS/ALAT clinical practice guideline, 2022 [PMID: 35486072].
definition of progression in IPF according to pubmed
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PubMed idiopathic pulmonary fibrosis disease progression definition FVC 10% criteria
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PMID: 35486072
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https://pmc.ncbi.nlm.nih.gov/articles/PMC9851481/
For idiopathic pulmonary fibrosis (IPF), there is no separate formal ATS/ERS definition of “progressive pulmonary fibrosis”. The 2022 PPF definition specifically applies to fibrotic interstitial lung disease other than IPF, because IPF is itself intrinsically progressive.
In clinical practice and many IPF studies, disease progression is generally identified by one or more of:
- Relative decline in FVC ≥10% over approximately 6-12 months. This is the most established marker and is associated with worse prognosis.
- A smaller relative FVC decline of 5-9%, particularly when accompanied by worsening symptoms, increased fibrosis on HRCT, reduced exercise capacity, or a meaningful DLCO decline.
- Worsening dyspnea or cough
- Radiological progression of fibrosis on HRCT, such as increased reticulation, traction bronchiectasis, or honeycombing.
- In some trial composite endpoints: FVC decline ≥10%, death, respiratory hospitalization, acute exacerbation, or lung transplantation.
Before labeling IPF as progressing, exclude other explanations for deterioration, including infection, acute exacerbation, pulmonary embolism, heart failure, pulmonary hypertension, drug toxicity, or a technically unreliable pulmonary-function test.
The key distinction is:
- PPF criteria: ≥2 of symptoms, physiological decline, and radiological progression within 1 year, and apply to non-IPF ILD.
- IPF progression: no single universally mandated composite definition, but a relative FVC decline ≥10% is the most widely accepted objective marker.
The 2022 ATS/ERS/JRS/ALAT guideline explicitly defines PPF only in ILD other than IPF: Raghu et al., 2022, PMID: 35486072.
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