ArticleBMC pulmonary medicine2014
Development of microscopic polyangiitis-related pulmonary fibrosis in a patient with autoimmune pulmonary alveolar proteinosis.
Article in BMC pulmonary medicine, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed, 10 citations in OpenAlex.
- Rare case of autoimmune pulmonary alveolar proteinosis with ANCA positivity.BMC pulmonary medicine · 2026Article
- A Rare Case of Autoimmune Pulmonary Alveolar Proteinosis Developing During the Course of Eosinophilic Granulomatosis With Polyangiitis.Respirology case reports · 2025Article
- Autoimmune pulmonary alveolar proteinosis developed during treatment for systemic sclerosis: a case report.BMC pulmonary medicine · 2025Article
- Clinical characteristics of pulmonary alveolar proteinosis.Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences · 2021Article
- Natural Autoantibodies in Chronic Pulmonary Diseases.International journal of molecular sciences · 2020Review
- Pathological features of explant lungs with fibrosis in autoimmune pulmonary alveolar proteinosis.Respirology case reports · 2017Article
- Pulmonary melanoma and "crazy paving" patterns in chest images: a case report and literature review.BMC cancer · 2016Review
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Authors and funding
16 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAutoimmune pulmonary alveolar proteinosis (aPAP) is a rare lung disease caused by the autoantibody against granulocyte-macrophage colony stimulating factor (GM-CSF). The clinical course of aPAP is variable; in severe cases, patients develop lethal respiratory failure due to pulmonary fibrosis. However, the pathogenesis of pulmonary fibrosis in aPAP has never been delineated. CASE PRESENTATION: Here, we describe a rare case of aPAP that was subsequently complicated by microscopic polyangiitis-related pulmonary fibrosis. The patient was a 75-year-old Japanese man diagnosed with aPAP based on the crazy-paving appearance on high-resolution computed tomography (HRCT), "milky" appearance of broncho-alveolar lavage fluid (BALF), and elevated serum levels of the anti-GM-CSF antibody. The patient was followed-up without aPAP-specific treatment for 3 years. During this period, both hematuria and proteinuria appeared; in addition, serum myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) turned positive and increased markedly. The second BAL performed one year after the diagnosis, showed that the "milky" appearance had resolved. The HRCT showed that fibrotic changes had developed and that the crazy-paving appearance had disappeared. These data suggest an association between pulmonary fibrosis that developed during the natural course of aPAP and ANCA-related systemic vasculitis.
conclusionThis is the first case report that suggests the existence of a pathogenetic relationship between ANCA-associated systemic vasculitis and aPAP-related pulmonary fibrosis. The link between ANCA-associated systemic vasculitis and aPAP-related pulmonary fibrosis requires further investigation.
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