Eosinophilic granulomatosis with polyangiitis (eGPA) is a small- and medium-sized-vessel vasculitis with multi-organ manifestations. Given the rarity of eGPA, patients are often misdiagnosed for decades and may initially present with life-threatening manifestations of late-stage disease. Therefore, it is important to raise awareness of this condition and its associated signs and symptoms. This case report serves to describe a classic presentation of a patient with eGPA, as well as to delineate the diagnostic workup, acute management, and early outpatient follow-up required.


Given the life-threatening complications of this rare condition, it is important to raise awareness about eGPA. This is a classic presentation of eGPA in a patient with a decades-long asthma history, eosinophilia, new lung and gastrointestinal manifestations, and systemic symptoms. His serologies were significant for positive C-ANCA of anti-PR-3 specificity. His clinical course included screening for gastrointestinal, renal, and cardiac involvement as well as initiating steroid therapy, plasmapheresis, and rituximab.