ArticleFrontiers in medicine2025
Hyaluronan in the pathogenesis of lung fibrosis associated to autoimmune pulmonary alveolar proteinosis (aPAP).
Article in Frontiers in medicine, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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1 citing paper in PubMed.
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Authors and funding
11 authors.
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No grant is acknowledged in the PubMed record.
Abstract
Introduction: Pulmonary fibrosis is a rare event occurring in patients with autoimmune Pulmonary Alveolar Proteinosis (aPAP). The accumulation of intra-alveolar material may promote proinflammatory and profibrotic pathways. Aim: To investigate serum biomarkers as prognostic factor of lung fibrosis in aPAP. Methods: We performed the ELF™ test (Siemens), which provides a score of fibrosis based on quantitative measurements of hyaluronic acid (HA), amino-terminal propeptide of type III procollagen (PIIINP), and tissue inhibitor of metalloproteinase 1 (TIMP-1) from serum of 21 aPAP patients, collected at diagnosis. Results: In this retrospective cohort study, we analyzed the serum samples collected at aPAP diagnosis from10 patients (PAP-FIB) showing lung fibrosis evidence on chest HRTC and 11 patients (PAP) who did not develop lung fibrosis within at least 9 years from aPAP diagnosis. Both PAP-FIB and PAP groups exhibited ELF scores above the risk threshold (>7.7), with significantly higher values in PAP-FIB (mean ELF score: 9.19 ± 0.75 vs. 8.52 ± 0.46, Conclusion: Our results seem to indicate that a robust HA production contributes to the chronic inflammatory and micro-injury to the alveolar epithelium. The high specificity of HA highlights its utility as a prognostic biomarker of aPAP-associated fibrosis.
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