ArticleERJ open research2021
Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia.
Article in ERJ open research, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 21 citations in OpenAlex.
- A Multicenter Registry for Rare Interstitial Lung Diseases in Korea: Baseline Characteristics and Clinical Outcomes.Tuberculosis and respiratory diseases · 2026Article
- Pleuroparenchymal Fibroelastosis With a Nodular Shadow Considered To Be Rounded Atelectasis: A Case Report.Respirology case reports · 2026Article
- Early pleuroparenchymal fibroelastosis mimicking lung malignancy: A case report.World journal of clinical cases · 2025Article
- Korean Guidelines for the Diagnosis and Management of Interstitial Lung Disease: Other Forms of Interstitial Lung Disease.Tuberculosis and respiratory diseases · 2025Article
- Baricitinib for Acute Exacerbation in Idiopathic Interstitial Pneumonias: A Preliminary Study Using Propensity Score Matching.Respiration; international review of thoracic diseases · 2025Article
- Rare interstitial lung diseases: a narrative review.Journal of thoracic disease · 2024Review
- Proposed Clinical Algorithm for Pleuroparenchymal Fibroelastosis (PPFE).Journal of clinical medicine · 2024Review
- Pulmonary fibroelastosis - A review.Matrix biology : journal of the International Society for Matrix Biology · 2023Review
- Assessment of malnutrition-related risk in patients with idiopathic pleuroparenchymal fibroelastosis.ERJ open research · 2023Article
- The world of rare interstitial lung diseases.European respiratory review : an official journal of the European Respiratory Society · 2023Review
- Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease.Turkish journal of medical sciences · 2022Article
- Nintedanib in idiopathic and secondary pleuroparenchymal fibroelastosis.Orphanet journal of rare diseases · 2021Article
- A proposed prognostic prediction score for pleuroparenchymal fibroelastosis.Respiratory research · 2021Article
- Effects of pulmonary rehabilitation therapy combined with conventional drugs on BODE and pulmonary function indexes in elderly patients with interstitial pneumonia.Pakistan journal of medical sciencesArticle
Corrections and comments
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Authors and funding
10 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThere are no established therapeutic options available for idiopathic pleuroparenchymal fibroelastosis (IPPFE) apart from supportive care and lung transplantation. Furthermore, it is known that IPPFE with a usual interstitial pneumonia (UIP) pattern and lower lobe predominance is a disease entity distinct from idiopathic pulmonary fibrosis (IPF). To our knowledge, few studies are available that report on the efficacy of antifibrotic agents for IPPFE with UIP.
aimThe aim of this study was to compare the efficacy of antifibrotic agents between IPPFE with UIP and typical IPF in real-world clinical practice. PATIENTS AND
methodsA retrospective analysis was performed on the medical records of all patients at two interstitial lung disease centres. Sixty-four patients were diagnosed as having IPPFE with UIP and 195 patients were diagnosed with typical IPF. We compared the efficacy of antifibrotic agents between these two groups.
resultsSurvival time was significantly shorter in the patients with IPPFE with UIP. Some 125 patients were administered antifibrotic agents for over 6 months (34 with IPPFE with UIP and 91 with typical IPF). Reduced forced vital capacity (FVC) 6 months after treatment with antifibrotic agents was significantly greater in the IPPFE with UIP group than in those in the typical IPF group. Moreover, the change in % predicted FVC was significantly greater during the follow-up in patients with IPPFE with UIP compared with those with typical IPF.
conclusionsThe efficacy of antifibrotic agents was limited in patients with IPPFE with UIP. Thus, IPPFE with UIP remains a fatal and progressive disease.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.