A Rare Case of Autoimmune Pulmonary Alveolar Proteinosis Developing During the Course of Eosinophilic Granulomatosis With Polyangiitis
ABSTRACT Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti‐neutrophil cytoplasmic antibody (ANCA)‐associated vasculitis characterised by asthma, eosinophilia, and systemic inflammation, often involving the lungs. We present the case of a 47‐year‐old woman with EGPA who developed progre...
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Wiley
2025-05-01
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| Series: | Respirology Case Reports |
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| Online Access: | https://doi.org/10.1002/rcr2.70219 |
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| author | Yoichi Dotake Kentaro Tanaka Shiro Fujisaki Kenichi Shimobaba Hirotoshi Kuroiwa Midori Satomura Hiromi Matsuyama Koichi Takagi Hideo Mitsuyama Hiromasa Inoue |
| author_facet | Yoichi Dotake Kentaro Tanaka Shiro Fujisaki Kenichi Shimobaba Hirotoshi Kuroiwa Midori Satomura Hiromi Matsuyama Koichi Takagi Hideo Mitsuyama Hiromasa Inoue |
| author_sort | Yoichi Dotake |
| collection | DOAJ |
| description | ABSTRACT Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti‐neutrophil cytoplasmic antibody (ANCA)‐associated vasculitis characterised by asthma, eosinophilia, and systemic inflammation, often involving the lungs. We present the case of a 47‐year‐old woman with EGPA who developed progressive ground‐glass opacities and a crazy‐paving pattern on chest computed tomography (CT). Bronchoalveolar lavage revealed milky fluid, and transbronchial lung biopsy showed periodic acid‐Schiff (PAS)‐positive eosinophilic granular material. Elevated anti‐granulocyte‐macrophage colony‐stimulating factor (GM–CSF) antibodies confirmed a diagnosis of autoimmune pulmonary alveolar proteinosis (aPAP). Corticosteroid tapering initially led to EGPA relapse, which was successfully controlled with mepolizumab, enabling further steroid reduction. Following this, the radiological findings of aPAP showed gradual improvement. In rare cases, it is known that autoimmune diseases such as vasculitis can be complicated by aPAP. This case highlights the importance of individualised immunomodulatory treatment and close imaging follow‐up in patients with overlapping autoimmune conditions. |
| format | Article |
| id | doaj-art-c13d73b643394889ae707e5a1f039e4f |
| institution | DOAJ |
| issn | 2051-3380 |
| language | English |
| publishDate | 2025-05-01 |
| publisher | Wiley |
| record_format | Article |
| series | Respirology Case Reports |
| spelling | doaj-art-c13d73b643394889ae707e5a1f039e4f2025-08-20T03:05:22ZengWileyRespirology Case Reports2051-33802025-05-01135n/an/a10.1002/rcr2.70219A Rare Case of Autoimmune Pulmonary Alveolar Proteinosis Developing During the Course of Eosinophilic Granulomatosis With PolyangiitisYoichi Dotake0Kentaro Tanaka1Shiro Fujisaki2Kenichi Shimobaba3Hirotoshi Kuroiwa4Midori Satomura5Hiromi Matsuyama6Koichi Takagi7Hideo Mitsuyama8Hiromasa Inoue9Department of Pulmonary Medicine Graduate School of Medical and Dental Sciences, Kagoshima University Kagoshima JapanDepartment of Pulmonary Medicine Graduate School of Medical and Dental Sciences, Kagoshima University Kagoshima JapanDepartment of Pulmonary Medicine Graduate School of Medical and Dental Sciences, Kagoshima University Kagoshima JapanDepartment of Pulmonary Medicine Graduate School of Medical and Dental Sciences, Kagoshima University Kagoshima JapanDepartment of Pulmonary Medicine Graduate School of Medical and Dental Sciences, Kagoshima University Kagoshima JapanDepartment of Pulmonary Medicine Graduate School of Medical and Dental Sciences, Kagoshima University Kagoshima JapanDepartment of Pulmonary Medicine Graduate School of Medical and Dental Sciences, Kagoshima University Kagoshima JapanDepartment of Pulmonary Medicine Graduate School of Medical and Dental Sciences, Kagoshima University Kagoshima JapanDepartment of Pulmonary Medicine Graduate School of Medical and Dental Sciences, Kagoshima University Kagoshima JapanDepartment of Pulmonary Medicine Graduate School of Medical and Dental Sciences, Kagoshima University Kagoshima JapanABSTRACT Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti‐neutrophil cytoplasmic antibody (ANCA)‐associated vasculitis characterised by asthma, eosinophilia, and systemic inflammation, often involving the lungs. We present the case of a 47‐year‐old woman with EGPA who developed progressive ground‐glass opacities and a crazy‐paving pattern on chest computed tomography (CT). Bronchoalveolar lavage revealed milky fluid, and transbronchial lung biopsy showed periodic acid‐Schiff (PAS)‐positive eosinophilic granular material. Elevated anti‐granulocyte‐macrophage colony‐stimulating factor (GM–CSF) antibodies confirmed a diagnosis of autoimmune pulmonary alveolar proteinosis (aPAP). Corticosteroid tapering initially led to EGPA relapse, which was successfully controlled with mepolizumab, enabling further steroid reduction. Following this, the radiological findings of aPAP showed gradual improvement. In rare cases, it is known that autoimmune diseases such as vasculitis can be complicated by aPAP. This case highlights the importance of individualised immunomodulatory treatment and close imaging follow‐up in patients with overlapping autoimmune conditions.https://doi.org/10.1002/rcr2.70219asthmaeosinophilic granulomatosis with polyangiitispulmonary alveolar proteinosis |
| spellingShingle | Yoichi Dotake Kentaro Tanaka Shiro Fujisaki Kenichi Shimobaba Hirotoshi Kuroiwa Midori Satomura Hiromi Matsuyama Koichi Takagi Hideo Mitsuyama Hiromasa Inoue A Rare Case of Autoimmune Pulmonary Alveolar Proteinosis Developing During the Course of Eosinophilic Granulomatosis With Polyangiitis Respirology Case Reports asthma eosinophilic granulomatosis with polyangiitis pulmonary alveolar proteinosis |
| title | A Rare Case of Autoimmune Pulmonary Alveolar Proteinosis Developing During the Course of Eosinophilic Granulomatosis With Polyangiitis |
| title_full | A Rare Case of Autoimmune Pulmonary Alveolar Proteinosis Developing During the Course of Eosinophilic Granulomatosis With Polyangiitis |
| title_fullStr | A Rare Case of Autoimmune Pulmonary Alveolar Proteinosis Developing During the Course of Eosinophilic Granulomatosis With Polyangiitis |
| title_full_unstemmed | A Rare Case of Autoimmune Pulmonary Alveolar Proteinosis Developing During the Course of Eosinophilic Granulomatosis With Polyangiitis |
| title_short | A Rare Case of Autoimmune Pulmonary Alveolar Proteinosis Developing During the Course of Eosinophilic Granulomatosis With Polyangiitis |
| title_sort | rare case of autoimmune pulmonary alveolar proteinosis developing during the course of eosinophilic granulomatosis with polyangiitis |
| topic | asthma eosinophilic granulomatosis with polyangiitis pulmonary alveolar proteinosis |
| url | https://doi.org/10.1002/rcr2.70219 |
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