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When Eosinophils Attack: A Rare Pulmonary Manifest ...
When Eosinophils Attack: A Rare Pulmonary Manifestation of Eosinophilic Granulomatosis With Polyangitis
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This case report describes eosinophilic granulomatosis with polyangiitis (EGPA), a rare autoimmune vasculitis of small-to-medium vessels, presenting unusually as a pleural effusion and pneumothorax. EGPA typically begins with allergic symptoms such as asthma, rhinitis, and nasal polyps, followed by peripheral eosinophilia and then vasculitic organ involvement. Because pleural effusion is an uncommon initial manifestation, the diagnosis can be difficult to recognize.<br /><br />An 80-year-old man with a history of scalp angiosarcoma, arterial thrombosis on warfarin, and eosinophilic esophagitis was found to have a new right pneumothorax and moderate pleural effusion on pre-biopsy CT imaging. He also had chronic productive cough, intermittent hemoptysis, and significant weight loss. Laboratory studies showed anemia, eosinophilia, elevated inflammatory markers, and high IgE. Imaging revealed cavitary lung lesions and nodules that were new compared with prior scans. Pleural fluid was exudative and strongly eosinophilic, while cytology, cultures, and infectious workup were negative.<br /><br />Because the hydropneumothorax persisted, the patient underwent wedge resection. Pathology showed chronic interstitial inflammation with prominent eosinophilic infiltration, organizing pneumonia, non-caseating granulomas, and intra-alveolar hemorrhage, without malignancy or infection. Although ANCA and other serologies were negative, the combination of peripheral eosinophilia and biopsy-proven extravascular eosinophilic inflammation met 2022 ACR/EULAR criteria for EGPA.<br /><br />The authors emphasize that EGPA can present atypically and that early recognition is critical, as delayed diagnosis may lead to severe complications. They note that the patient likely would have benefited from timely high-dose corticosteroid therapy, but his condition worsened before confirmation of the diagnosis.
Asset Subtitle
Kunal Jain
Meta Tag
Author List
Jennifer Mao, Kunal Jain, Mahnoor Khalid, Sameer Shaik, Zaid Aslam
Category
Clinical Vignettes
Concept
Eosinophilic Granulomatosis with Polyangiitis
Concept
Eosinophilia
Concept
Extravascular Eosinophilic Inflammation
Concept
Disease Progression
Concept
Pulmonary Findings
Distinguished
Non-Finalist
Presenter Organization
Texas Health Resources
Presenting Author
Kunal Jain
Track
Adult
Keywords
eosinophilic granulomatosis with polyangiitis
EGPA
pleural effusion
pneumothorax
eosinophilia
autoimmune vasculitis
cavitary lung lesions
eosinophilic inflammation
ANCA-negative
corticosteroid therapy
Eosinophilic Granulomatosis with Polyangiitis
Eosinophilia
Extravascular Eosinophilic Inflammation
Disease Progression
Pulmonary Findings
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