ArticleAmerican journal of respiratory and critical care medicine2008
Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan.
Article in American journal of respiratory and critical care medicine, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 225 papers, 4 of them syntheses that pooled it.
What it found
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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
225 citing papers in PubMed, 4 syntheses or guidelines pooled it, 479 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
- 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
- A Hereditary Pulmonary Alveolar Proteinosis Caused by a Novel Hemizygous Variation of the CSF2RA Gene Case Report and Literature Review.Molecular genetics & genomic medicine · 2025Pooled it
- Better approach for autoimmune pulmonary alveolar proteinosis treatment: inhaled or subcutaneous granulocyte-macrophage colony-stimulating factor: a meta-analyses.Respiratory research · 2018Pooled it
- Inhaled recombinant GM-CSF reduces the need for whole lung lavage and improves gas exchange in autoimmune pulmonary alveolar proteinosis patients.The European respiratory journal · 2024Trial
- Inhaled Molgramostim Therapy in Autoimmune Pulmonary Alveolar Proteinosis.The New England journal of medicine · 2020Trial
- Inhaled granulocyte-macrophage colony stimulating factor for mild-to-moderate autoimmune pulmonary alveolar proteinosis - a six month phase II randomized study with 24 months of follow-up.Orphanet journal of rare diseases · 2020Trial
- Granulocyte-macrophage colony-stimulating factor (GM-CSF) as a therapeutic target in psoriasis: randomized, controlled investigation using namilumab, a specific human anti-GM-CSF monoclonal antibody.The British journal of dermatology · 2019Trial
- Trial
- Confocal laser endomicroscopy for diagnosis and monitoring of pulmonary alveolar proteinosis.Journal of bronchology & interventional pulmonology · 2015Trial
- Efficacy and safety of mavrilimumab in subjects with rheumatoid arthritis.Annals of the rheumatic diseases · 2013Trial
- An open-label trial of rituximab therapy in pulmonary alveolar proteinosis.The European respiratory journal · 2011Trial
- Pulmonary Alveolar Proteinosis: Rethinking Surfactant Homeostasis Through Alveolar Macrophage Lipid-Handling Dysfunction.Arteriosclerosis, thrombosis, and vascular biology · 2026Review
- Lung transplantation for pulmonary alveolar proteinosis: a retrospective international multicentre analysis.The European respiratory journal · 2026Article
- Pulmonary nocardiosis in patients with underlying pulmonary alveolar proteinosis: a case series and review of the literature.BMC pulmonary medicine · 2026Review
- Affinity- and epitope-dependent pathogenicity of GM-CSF autoantibodies in patients with autoimmune pulmonary alveolar proteinosis.Nature communications · 2026Article
- Phagocytotic impairment of tissue-resident alveolar macrophages by diesel particulates drives pulmonary surfactant accumulation.Advanced biotechnology · 2026Article
- Successful Whole-Lung Lavage in Anti-GM-CSF-Negative Pulmonary Alveolar Proteinosis: A Case Report.The American journal of case reports · 2026Article
- Anti-granulocyte macrophage colony-stimulating factor autoantibodies: A rising cause of infectious diseases.PLoS pathogens · 2026Article
- A long-term observational study on autoimmune pulmonary alveolar proteinosis revealed a sustained and generalized decrease in serum autoantibody levels.Orphanet journal of rare diseases · 2026Observational
165 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
21 authors at 14 institutions in 3 countries.
Funding
Abstract
rationaleAcquired pulmonary alveolar proteinosis (PAP) is a syndrome characterized by pulmonary surfactant accumulation occurring in association with granulocyte/macrophage colony-stimulating factor autoantibodies (autoimmune PAP) or as a consequence of another disease (secondary PAP). Because PAP is rare, prior reports were based on limited patient numbers or a synthesis of historical data.
objectivesTo describe the epidemiologic, clinical, physiologic, and laboratory features of autoimmune PAP in a large, contemporaneous cohort of patients with PAP.
methodsOver 6 years, 248 patients with PAP were enrolled in a Japanese national registry, including 223 with autoimmune PAP. MEASUREMENTS AND MAIN
resultsAutoimmune PAP represented 89.9% of cases and had a minimum incidence and prevalence of 0.49 and 6.2 per million, respectively. The male to female ratio was 2.1:1, and the median age at diagnosis was 51 years. A history of smoking occurred in 56%, and dust exposure occurred in 23%; instances of familial onset did not occur. Dyspnea was the most common presenting symptom, occurring in 54.3%. Importantly, 31.8% of patients were asymptomatic and were identified by health screening. Intercurrent illnesses, including infections, were infrequent. A disease severity score reflecting the presence of symptoms and degree of hypoxemia correlated well with carbon monoxide diffusing capacity and serum biomarkers, less well with pulmonary function, and not with granulocyte/macrophage colony-stimulating factor autoantibody levels or duration of disease.
conclusionsAutoimmune PAP had an incidence and prevalence higher than previously reported and was not strongly linked to smoking, occupational exposure, or other illnesses. The disease severity score and biomarkers provide novel and potentially useful outcome measures in 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.