CONTEXT: Relatively small scale retrospective analysis of IPF patients in Japan reveals outcomes are poor for patients developing progressive fibrosing interstitial lung disease (PF-ILD) other than idiopathic pulmonary fibrosis (IPF).
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1. “Patients can develop a so-called “progressive fibrosing phenotype” (PF-ILD) that causes lung fibrosis, rapid lung function decline, and a person’s life quality and lifespan — similar to IPF, the most common ILD and the most typical example of PF-ILD.”
2. “Outcomes are as poor in people with progressive fibrosing interstitial lung disease (PF-ILD) other than idiopathic pulmonary fibrosis (IPF) as in those with IPF, according to a Japanese real-world study.”
3. “Researchers also found that worse lung function at a first assessment and the presence of fibrotic patterns in the lungs characteristic of IPF could raise the risk of disease progression in non-IPF ILD.”
4. “People with PF-ILD other than IPF showed significantly worse lung function at the first and second exams than did those without progressive disease, and more frequently exhibited the usual interstitial pneumonia-like fibrotic pattern — characteristic of IPF — in their lungs (54.5% vs. 13%).”
5. “Further analysis suggested that low %FVC at a first lung function assessment and the presence of IPF-characteristic fibrotic pattern might be risk factors of progressive disease in people with non-IPF ILD.”
Source URL: https://pulmonaryfibrosisnews.com/2021/04/19/outcomes-poor-non-ipf-pf-ild-real-world-study-japan/