ReviewBreathe (Sheffield, England)2020
Pulmonary alveolar proteinosis: from classification to therapy.
Review in Breathe (Sheffield, England), 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers, 3 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
37 citing papers in PubMed, 3 syntheses or guidelines pooled it, 58 citations in OpenAlex.
- Pooled it
- The burden of autoimmune pulmonary alveolar proteinosis: a systematic review.European respiratory review : an official journal of the European Respiratory Society · 2026Pooled it
- Efficacy of recombinant human GM-CSF compared to placebo for autoimmune pulmonary alveolar proteinosis: a systematic review and meta-analysis.BMC pulmonary medicine · 2026Pooled it
- CRISPR screening identifies DTX4 governing alveolar macrophage cholesterol efflux in pulmonary alveolar proteinosis.JCI insight · 2026Article
- Targeting the PPAR-γ-ABC transporter axis ameliorates secondary pulmonary alveolar proteinosis induced by indium oxide nanoparticles.Particle and fibre toxicology · 2026Article
- Successful Whole-Lung Lavage in Anti-GM-CSF-Negative Pulmonary Alveolar Proteinosis: A Case Report.The American journal of case reports · 2026Article
- Clinical characterization of 39 patients with autoimmune pulmonary alveolar proteinosis.The Journal of international medical research · 2026Article
- Article
- A Rare Case of Pulmonary Alveolar Proteinosis.Cureus · 2025Article
- Hereditary pulmonary alveolar proteinosis in a 5-year-old child: Diagnostic insights and therapeutic approach.Radiology case reports · 2025Article
- Position-Dependent Lung Shadow Movement.Respirology case reports · 2025Article
- Case Report: Innovative anesthetic approaches for whole lung lavage in an infant with pulmonary alveolar proteinosis.Frontiers in pediatrics · 2025Article
- Autoimmunity against cytokines: Double strike in autoimmune disease, a historical perspectiveBiomedica : revista del Instituto Nacional de Salud · 2024Review
- Autoimmune pulmonary alveolar proteinosis presenting as localized multifocal GGOs: A case report.Radiology case reports · 2024Article
- Clinical approach for pulmonary alveolar proteinosis in children.World journal of clinical cases · 2024Article
- A Comprehensive Outlook on Pulmonary Alveolar Proteinosis-A Review.International journal of molecular sciences · 2024Review
- Pulmonary alveolar proteinosis following severe COVID - 19 infection: A case report.Respiratory medicine case reports · 2024Article
- Common Mutations in the Surfactant Protein-C Gene in Iranian Patients with Diffuse Parenchymal Lung Disease.Tanaffos · 2024Article
- [Congenital pulmonary alveolar proteinosis in a neonate].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2023Article
- Unexpected diffuse lung lesions in a patient with pulmonary alveolar proteinosis: A case report.World journal of clinical cases · 2023Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Pulmonary alveolar proteinosis (PAP) is a rare respiratory syndrome characterised by the accumulation of surfactant lipoproteins within the alveoli. According to various pathogenetic mechanisms and aetiologies, PAP is classified as primary, secondary or congenital. Primary PAP is led by a granulocyte-macrophage colony-stimulating factor (GM-CSF) signalling disruption; the autoimmune form is driven by the presence of anti GM-CSF autoantibodies and represents 90% of all the PAP cases; and the hereditary form is the result of mutations in genes encoding GM-CSF receptor. Secondary PAP is associated with various diseases causing a reduction in function and/or number of alveolar macrophages. Congenital PAP emerges as a consequence of corrupted surfactant production, due to mutations in surfactant proteins or lipid transporter, or mutations affecting lung development. The clinical manifestations are various, ranging from insidious onset to acute or progressive respiratory failure, including premature death within the first days of life in neonates with congenital surfactant production disorders. The diagnostic workup includes clinical and radiological assessment (respiratory function test, high-resolution chest computed tomography), laboratory tests (anti-GM-CSF autoantibodies dosage, GM-CSF serum level and GM-CSF signalling test), and genetic tests. Whole-lung lavage is the current gold standard of care of PAP; however, the therapeutic approach depends on the pathogenic form and disease severity, including GM-CSF augmentation strategies in autoimmune PAP and other promising new treatments. EDUCATIONAL
aimsTo update knowledge about a rare respiratory syndrome, pulmonary alveolar proteinosis, in order to promote early diagnosis and correct management.To highlight recent treatment options based on pathogenesis and disease severity.
Identifiers
What OpenQuestion holds
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.