ArticleNature communications2018
Statin as a novel pharmacotherapy of pulmonary alveolar proteinosis.
Article in Nature communications, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 52 papers, 1 of them a synthesis that pooled it.
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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.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
52 citing papers in PubMed, 1 synthesis or guideline pooled it, 84 citations in OpenAlex.
- The burden of autoimmune pulmonary alveolar proteinosis: a systematic review.European respiratory review : an official journal of the European Respiratory Society · 2026Pooled it
- Inhaled Molgramostim Therapy in Autoimmune Pulmonary Alveolar Proteinosis.The New England journal of medicine · 2020Trial
- CRISPR screening identifies DTX4 governing alveolar macrophage cholesterol efflux in pulmonary alveolar proteinosis.JCI insight · 2026Article
- Isoleucyl-tRNA synthetase 1 mutation impairs pulmonary surfactant homeostasis by disrupting alveolar macrophage function.Respiratory research · 2026Article
- Lipid accumulation in tuberculosis granulomas inhibits macrophage-CD4Nature microbiology · 2026Article
- Clinical characterization of 39 patients with autoimmune pulmonary alveolar proteinosis.The Journal of international medical research · 2026Article
- Significant but Temporary Efficacy of Statin for a Patient With Severe Autoimmune Pulmonary Alveolar Proteinosis: A Case Report.Respirology case reports · 2026Article
- Review
- Cholesterol Regulates Airway Epithelial Cell Differentiation by Inhibiting p53 Nuclear Translocation.International journal of molecular sciences · 2025Article
- The dark side of pulmonary alveolar proteinosis.Multidisciplinary respiratory medicine · 2025Article
- Alveolar macrophages: guardians of the alveolar lipid galaxy.Current opinion in lipidology · 2025Review
- Pediatric Pulmonology 2024 Year in Review: Rare and Diffuse Lung Disease.Pediatric pulmonology · 2025Review
- Phenotypic heterogeneity in mortality and prognosis of pulmonary alveolar proteinosis: a large-scale, global pooled analysis of individual-level data.Orphanet journal of rare diseases · 2025Article
- Position-Dependent Lung Shadow Movement.Respirology case reports · 2025Article
- The healthcare burden of pulmonary alveolar proteinosis (PAP).Orphanet journal of rare diseases · 2025Article
- Pathogenesis-driven treatment of primary pulmonary alveolar proteinosis.European respiratory review : an official journal of the European Respiratory Society · 2024Review
- A Comprehensive Outlook on Pulmonary Alveolar Proteinosis-A Review.International journal of molecular sciences · 2024Review
- Cancer immunometabolism: advent, challenges, and perspective.Molecular cancer · 2024Review
- Ten-year experience of whole lung lavage in pediatric Pulmonary Alveolar Proteinosis.Klinische Padiatrie · 2024Article
- Pulmonary Alveolar Proteinosis and new therapeutic concepts.Klinische Padiatrie · 2024Article
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Authors and funding
14 authors at 3 institutions in 1 country.
Funding
Abstract
Pulmonary alveolar proteinosis (PAP) is a syndrome of reduced GM-CSF-dependent, macrophage-mediated surfactant clearance, dysfunctional foamy alveolar macrophages, alveolar surfactant accumulation, and hypoxemic respiratory failure for which the pathogenetic mechanism is unknown. Here, we examine the lipids accumulating in alveolar macrophages and surfactant to define the pathogenesis of PAP and evaluate a novel pharmacotherapeutic approach. In PAP patients, alveolar macrophages have a marked increase in cholesterol but only a minor increase in phospholipids, and pulmonary surfactant has an increase in the ratio of cholesterol to phospholipids. Oral statin therapy is associated with clinical, physiological, and radiological improvement in autoimmune PAP patients, and ex vivo statin treatment reduces cholesterol levels in explanted alveolar macrophages. In Csf2rb-/- mice, statin therapy reduces cholesterol accumulation in alveolar macrophages and ameliorates PAP, and ex vivo statin treatment increases cholesterol efflux from macrophages. These results support the feasibility of statin as a novel pathogenesis-based pharmacotherapy of PAP.
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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.