A Case of Eosinophilic Granulomatosis with Polyangiitis Presenting with Mononeuritis Multiplex

Abstract

Background: Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg Strauss syndrome, is an uncommon vasculitis associated with antineutrophil cytoplasmic an-tibody (ANCA). The hallmarks of the disease are asthma, eosinophilia, and systemic vasculitis with varying degrees of neurological, cutaneous, cardiac, gastrointestinal, and renal involvement. Diag-nosis is often difficult since the symptoms are diverse, and a number of differentials need to be ex-cluded. Case presentation: In this report, we describe a 60-year-old patient who presented with mononeuri-tis multiplex and a painful skin rash. A history of late-onset asthma, which was poorly controlled, led us to suspect EGPA. Laboratory data showed leukocytosis, eosinophilia (>10%), elevated ESR, CRP, and IgE, normal chest Xray, positive rheumatoid factor (RA), perinuclear anti-neutrophil cy-toplasmic antibody (p-ANCA), and evidence of extravascular eosinophils in histopathology report of skin biopsy. She was treated with methylprednisolone and cyclophosphamide pulse therapy with a satisfactory response. Conclusion: Diagnosis of EGPA requires a combination of clinical and histopathological findings to meet the diagnostic criteria. A history of poorly controlled or late-onset asthma may guide us to the diagnosis that is frequently overlooked. Due to the wide heterogeneity of EGPA patients' pheno-types, sharp, professional judgment is needed for early disease detection and treatment in order to avoid irreversible changes and poor outcomes.