ArticleRespiratory medicine2018
Functional associations of pleuroparenchymal fibroelastosis and emphysema with hypersensitivity pneumonitis.
Article in Respiratory medicine, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers, 1 of them a synthesis that pooled it.
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Who cites it
35 citing papers in PubMed, 1 synthesis or guideline pooled it, 77 citations in OpenAlex.
- Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline.American journal of respiratory and critical care medicine · 2020Guideline
- Quantitative assessment of fibroelastosis reveals distinct elastic and collagen fiber patterns in idiopathic and secondary pleuroparenchymal fibroelastosis.Virchows Archiv : an international journal of pathology · 2026Article
- Impact of older age on prognosis in hypersensitivity pneumonitis: A systematic review and meta-analysis.Experimental and therapeutic medicine · 2026Article
- Atypical Computed Tomography Findings of Non-fibrotic Hypersensitivity Pneumonia with Emphysema: A Case Report and Literature Review.Internal medicine (Tokyo, Japan) · 2026Review
- Korean Guidelines for the Diagnosis and Management of Interstitial Lung Disease: Other Forms of Interstitial Lung Disease.Tuberculosis and respiratory diseases · 2025Article
- Prognostic value of coexisting conditions and complications in pleuroparenchymal fibroelastosis: a single-center retrospective study.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2024Article
- Proposed Clinical Algorithm for Pleuroparenchymal Fibroelastosis (PPFE).Journal of clinical medicine · 2024Review
- Mortality surrogates in combined pulmonary fibrosis and emphysema.The European respiratory journal · 2024Article
- Multidisciplinary Approach to the Diagnosis of Idiopathic Interstitial Pneumonias: Focus on the Pathologist's Key Role.International journal of molecular sciences · 2024Review
- A Rare Case of Idiopathic Pleuro-Parenchymal Fibroelastosis Presenting With Bilateral Spontaneous Pneumothoraces.Cureus · 2024Article
- Article
- The world of rare interstitial lung diseases.European respiratory review : an official journal of the European Respiratory Society · 2023Review
- Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis.ERJ open research · 2023Article
- Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement.American journal of respiratory and critical care medicine · 2022Article
- Pleuroparenchymal fibroelastosis in rheumatoid arthritis-associated interstitial lung disease.Respiratory research · 2022Article
- Demystifying idiopathic interstitial pneumonia: time for more etiology-focused nomenclature in interstitial lung disease.Expert review of respiratory medicine · 2022Review
- Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias.Respiratory research · 2021Article
- Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment.EClinicalMedicine · 2021Article
- Pleuroparenchymal fibroelastosis: from diagnosis to pulmonary rehabilitation.BMJ case reports · 2021Article
- HRCT Diagnosis of Pleuroparenchymal fibroelastosis: Report of two cases.Radiology case reports · 2021Article
Corrections and comments
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Authors and funding
10 authors at 5 institutions in 4 countries.
Funding
Abstract
backgroundPleuroparenchymal fibroelastosis (PPFE) has been described in hypersensitivity pneumonitis (HP) yet its functional implications are unclear. Combined pulmonary fibrosis and emphysema (CPFE) has occasionally been described in never-smokers with HP, but epidemiological data regarding its prevalence is sparse. CTs in a large HP cohort were therefore examined to identify the prevalence and effects of PPFE and emphysema.
methods233 HP patients had CT extents of interstitial lung disease (ILD) and emphysema quantified to the nearest 5%. Lobar percentage pleural involvement of PPFE was quantified on a 4-point categorical scale: 0 = absent, 1 = affecting <10%, 2 = affecting 10-33%, 3 = affecting >33%. Marked PPFE reflected a total lung score of ≥3/18. Results were evaluated against FVC, DLco and mortality.
resultsMarked PPFE prevalence was 23% whilst 23% of never-smokers had emphysema. Following adjustment for patient age, gender, smoking status, and ILD and emphysema extents, marked PPFE independently linked to reduced baseline FVC (p = 0.0002) and DLco (p = 0.002) and when examined alongside the same covariates, independently linked to worsened survival (p = 0.01). CPFE in HP demonstrated a characteristic functional profile of artificial lung volume preservation and disproportionate DLco reduction. CPFE did not demonstrate a worsened outcome when compared to HP patients without emphysema beyond that explained by CT extents of ILD and emphysema.
conclusionsPPFE is not uncommon in HP, and is independently associated with impaired lung function and increased mortality. Emphysema was identified in 23% of HP never-smokers. CPFE appears not to link to a malignant microvascular phenotype as outcome is explained by ILD and emphysema extents.
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