Eosinophilic Granulomatosis with Polyangiitis (EGPA): Our Experience

Abstract

Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of several organs. We tried to analyze clinical phenotype and treatment in 3 patients diagnosed of EGPA at our hospital. Materials and Methods A retrospective analysis was made of 3 patients diagnosed of EGPA at Cardarelli Hospital from 2017 at 2023. Data regarding personal antecedents, clinical manifestations, analytical parameters, histology, and clinical course were collected. Results Of the 3 cases, 2 were women, 2 were diagnosed at an age between 51 and 80 years; one case to 22 years. All patients had antecedents of bronchial asthma and eosinophilia. Among clinical manifestations, women presented sensory-motor symmetric axonal neuropathy, two patients had fever and interstitial lung disease, the younger woman presented with pericarditis. One had non severe EGPA, two severe EGPA. For remission maintenance, in patients with severe EGPA, we used DMARDs, mepolizumab and glucocorticoids. In the patient with non-severe EGPA, only glucocorticoids in combination with mepolizumab. Discussion Our experience showed that EGPA patients should be offered the best care through interdisciplinary management and well-defined treatment-to-target approaches to achieve remission.