Overlap or Outlier? Granulomatosis With Polyangiitis With Eosinophilia: A Case Report and Diagnostic Insight
Granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA) are distinct yet overlapping forms of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, each with unique clinical features. GPA typically involves necrotizing granulomatous inflammation of the upper and lower respirat...
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| Format: | Article |
| Language: | English |
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SAGE Publishing
2025-07-01
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| Series: | Journal of Investigative Medicine High Impact Case Reports |
| Online Access: | https://doi.org/10.1177/23247096251363027 |
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| author | Sinen Tadesse Zeleke MD Syeda Ramsha Zaidi MD Ademsegd Isac Gebremedhen MD Melinda Becker MD |
| author_facet | Sinen Tadesse Zeleke MD Syeda Ramsha Zaidi MD Ademsegd Isac Gebremedhen MD Melinda Becker MD |
| author_sort | Sinen Tadesse Zeleke MD |
| collection | DOAJ |
| description | Granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA) are distinct yet overlapping forms of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, each with unique clinical features. GPA typically involves necrotizing granulomatous inflammation of the upper and lower respiratory tracts and is strongly associated with cytoplasmic ANCA (c-ANCA) and PR3-ANCA positivity, while EGPA is characterized by eosinophilia, asthma, and vasculitis. Rare cases of GPA can present with eosinophilia, complicating the distinction between these entities and raising the possibility of a GPA/EGPA overlap syndrome. We present the case of a 40-year-old female with a history of rheumatoid arthritis and recurrent pneumonia, admitted with worsening dyspnea, peripheral eosinophilia, and high titers of anti-PR3-ANCA. Bronchoalveolar lavage confirmed eosinophilic infiltration, leading to an initial diagnosis of GPA with eosinophilia. The patient responded well to corticosteroids, rituximab, and avacopan, with significant clinical improvement. This case underscores the importance of integrating clinical, serologic, and histopathologic findings when diagnosing ANCA-associated vasculitis, particularly in patients with eosinophilia and asthma-like symptoms. Recognizing GPA with eosinophilia as distinct from true GPA/EGPA overlap is crucial for prognosis and treatment decisions. |
| format | Article |
| id | doaj-art-831fe07efcce40cdb68d28b09228bc9a |
| institution | DOAJ |
| issn | 2324-7096 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Journal of Investigative Medicine High Impact Case Reports |
| spelling | doaj-art-831fe07efcce40cdb68d28b09228bc9a2025-08-20T03:09:32ZengSAGE PublishingJournal of Investigative Medicine High Impact Case Reports2324-70962025-07-011310.1177/23247096251363027Overlap or Outlier? Granulomatosis With Polyangiitis With Eosinophilia: A Case Report and Diagnostic InsightSinen Tadesse Zeleke MD0Syeda Ramsha Zaidi MD1Ademsegd Isac Gebremedhen MD2Melinda Becker MD3Marshall University Joan C. Edwards School of Medicine, Huntington, WV, USAMarshall University Joan C. Edwards School of Medicine, Huntington, WV, USAMarshall University Joan C. Edwards School of Medicine, Huntington, WV, USAMarshall University Joan C. Edwards School of Medicine, Huntington, WV, USAGranulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA) are distinct yet overlapping forms of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, each with unique clinical features. GPA typically involves necrotizing granulomatous inflammation of the upper and lower respiratory tracts and is strongly associated with cytoplasmic ANCA (c-ANCA) and PR3-ANCA positivity, while EGPA is characterized by eosinophilia, asthma, and vasculitis. Rare cases of GPA can present with eosinophilia, complicating the distinction between these entities and raising the possibility of a GPA/EGPA overlap syndrome. We present the case of a 40-year-old female with a history of rheumatoid arthritis and recurrent pneumonia, admitted with worsening dyspnea, peripheral eosinophilia, and high titers of anti-PR3-ANCA. Bronchoalveolar lavage confirmed eosinophilic infiltration, leading to an initial diagnosis of GPA with eosinophilia. The patient responded well to corticosteroids, rituximab, and avacopan, with significant clinical improvement. This case underscores the importance of integrating clinical, serologic, and histopathologic findings when diagnosing ANCA-associated vasculitis, particularly in patients with eosinophilia and asthma-like symptoms. Recognizing GPA with eosinophilia as distinct from true GPA/EGPA overlap is crucial for prognosis and treatment decisions.https://doi.org/10.1177/23247096251363027 |
| spellingShingle | Sinen Tadesse Zeleke MD Syeda Ramsha Zaidi MD Ademsegd Isac Gebremedhen MD Melinda Becker MD Overlap or Outlier? Granulomatosis With Polyangiitis With Eosinophilia: A Case Report and Diagnostic Insight Journal of Investigative Medicine High Impact Case Reports |
| title | Overlap or Outlier? Granulomatosis With Polyangiitis With Eosinophilia: A Case Report and Diagnostic Insight |
| title_full | Overlap or Outlier? Granulomatosis With Polyangiitis With Eosinophilia: A Case Report and Diagnostic Insight |
| title_fullStr | Overlap or Outlier? Granulomatosis With Polyangiitis With Eosinophilia: A Case Report and Diagnostic Insight |
| title_full_unstemmed | Overlap or Outlier? Granulomatosis With Polyangiitis With Eosinophilia: A Case Report and Diagnostic Insight |
| title_short | Overlap or Outlier? Granulomatosis With Polyangiitis With Eosinophilia: A Case Report and Diagnostic Insight |
| title_sort | overlap or outlier granulomatosis with polyangiitis with eosinophilia a case report and diagnostic insight |
| url | https://doi.org/10.1177/23247096251363027 |
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