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Biomarker and diagnostic research for idiopathic pleuroparenchymal fibroelastosis has been reported in the published literature.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
No clinical trials have been registered for idiopathic pleuroparenchymal fibroelastosis.
17 publications have been identified in PubMed for idiopathic pleuroparenchymal fibroelastosis. Research spans Diagnostic / Biomarker (18%), Review / Meta-Analysis (18%), and Case Report / Case Series (18%).
Research Type | Count | % of Total |
|---|---|---|
Testing and diagnosis research | 3 | 18% |
Data assembled from 3 of 12 sources · Last updated Sep 20, 2026, 3:07 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Research summaries
3 |
18% |
Patient case studies | 3 | 18% |
Laboratory research | 3 | 18% |
Disease patterns and progression | 3 | 18% |
Other research | 1 | 6% |
Clinical study results | 1 | 6% |
Kono M (2026). [PMID: 41780260](https://pubmed.ncbi.nlm.nih.gov/41780260/). *Respir Investig*. [Epidemiology / Natural History]
Hamada S (2026). [PMID: 41297378](https://pubmed.ncbi.nlm.nih.gov/41297378/). *Respir Investig*. [Case Report / Case Series]
Mori Y (2026). [PMID: 42140153](https://pubmed.ncbi.nlm.nih.gov/42140153/). *Respir Investig*. [Diagnostic / Biomarker]
Kinoshita Y (2026). [PMID: 42178527](https://pubmed.ncbi.nlm.nih.gov/42178527/). *BMC Pulm Med*. [Epidemiology / Natural History]
Kato M (2025). [PMID: 41290797](https://pubmed.ncbi.nlm.nih.gov/41290797/). *Sci Rep*. [Epidemiology / Natural History]
Suzuki Y (2025). [PMID: 39693846](https://pubmed.ncbi.nlm.nih.gov/39693846/). *Respir Investig*. [Review / Meta-Analysis]
Matsunashi A (2025). [PMID: 41177311](https://pubmed.ncbi.nlm.nih.gov/41177311/). *Respir Med*. [Basic Science / Preclinical]
Kinoshita Y (2025). [PMID: 39921067](https://pubmed.ncbi.nlm.nih.gov/39921067/). *Respir Med*. [Diagnostic / Biomarker]
Suzuki Y (2025). [PMID: 40491469](https://pubmed.ncbi.nlm.nih.gov/40491469/). *ERJ Open Res*. [Other]
Ohta H (2025). [PMID: 40239418](https://pubmed.ncbi.nlm.nih.gov/40239418/). *Respir Investig*. [Clinical Trial Publication]
AI-curated news mentioning idiopathic pleuroparenchymal fibroelastosis
Updated Jun 12, 2026
A recent study explores thoracic joint abnormalities and impaired motion in patients with idiopathic pleuroparenchymal fibroelastosis using CT imaging. This research may provide insights into the disease's impact on respiratory function.
A case report details the successful treatment of recurrent pneumothorax complicated by pleuroparenchymal fibroelastosis using pleurodesis with 50% glucose. This approach may offer insights into managing similar cases in rare disease contexts.
A study evaluates the reliability, validity, responsiveness, and minimal important difference of the St. George's Respiratory Questionnaire in patients with idiopathic pleuroparenchymal fibroelastosis. This research provides valuable insights into assessing respiratory health in this rare disease population.