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Beyond Classification: An Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Case
Lina Seffar1, Abderrahim Elktaibi2, Jalila El Bakkouri1
1Biology, Cheikh Khalifa International University Hospital, Mohammed VI University of Health Sciences, Casablanca, MAR.
Abstract:
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are usually classified as distinct entities, such as granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA). In everyday practice, however, some patients display overlapping features of both conditions, making classification and treatment decisions more challenging. We report a case of a 51-year-old man with late-onset asthma who presented with constitutional symptoms, purulent rhinosinusitis, hemoptysis, and arthralgia. Imaging demonstrated cavitary pulmonary nodules and nasal polyposis. Laboratory testing showed marked eosinophilia and positivity for proteinase 3 (PR3) c-ANCA. Nasal biopsy revealed necrotizing granulomatous inflammation rich in eosinophils. The patient received induction therapy with high-dose glucocorticoids and cyclophosphamide, followed by rituximab for maintenance, with clinical improvement and sustained remission. This case highlights the limitations of current classification frameworks and is compatible with a GPA-EGPA overlap phenotype (or spectrum). It underscores the value of an individualized approach guided by the predominant organ-threatening manifestations and the associated biological profile.
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