Kritika Bhattarai, Ashish Acharya, Santosh Basyal, Aashish Neupane, Shreema Basnet, Shobha Mandal, Jeevan Gautam, Anamol Acharya
Diagnosing EGPA can be challenging due to its variable clinical manifestations. Our case exhibited typical upper respiratory tract involvement; however, positive ANCA, p-ANCA, and MPO-ANCA findings were instrumental in confirming the diagnosis. Based on our literature review, we recommend that such cases be thoroughly evaluated and managed in hospital settings.
INTRODUCTION AND IMPORTANCE: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, multisystem vasculitic disorder characterized by asthma, eosinophilia, and necrotizing granulomatous inflammation with vasculitis affecting small- to medium-sized vessels. EGPA is classified as an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis.
PRESENTATION OF CASE: Here, we present a case of a 24-year-old male who came to our institution with complaints of progressive, bilateral, non-tender, non-erythematous nasal swelling since last year. Upon further inquiry, he was found to have asthma managed with inhaled short-acting bronchodilators and had recurrent episodes of rhinosinusitis in the past. After several supportive investigations, a diagnosis of EGPA with nasal polyposis was confirmed, and the patient was initially treated with intravenous corticosteroids, followed by oral doses for maintenance therapy after discharge.
DISCUSSION: In most cases, EGPA presents clinically with a history of asthma. Upper respiratory tract involvement occurs in 70-90% of cases, typically manifesting as rhinosinusitis and nasal polyposis. A positive ANCA - especially with a perinuclear pattern (p-ANCA) and antigen specificity for myeloperoxidase (MPO-ANCA) - is a hallmark of EGPA and aids in diagnosis. Treatment generally includes high-dose corticosteroids, with cyclophosphamide added in severe cases.
CONCLUSION: Diagnosing EGPA can be challenging due to its variable clinical manifestations. Our case exhibited typical upper respiratory tract involvement; however, positive ANCA, p-ANCA, and MPO-ANCA findings were instrumental in confirming the diagnosis. Based on our literature review, we recommend that such cases be thoroughly evaluated and managed in hospital settings.