Clinical characteristics of eosinophilic granulomatosis with polyangiitis

<p>Eosinophilic granulomatosis with polyangiitis (EGPA) is a subtype of anti-neutrophil cytoplasm antibody (ANCA) associated vasculitis. EGPA is often associated with asthma and allergic rhinitis, and its clinical features are similar to those of eosinophilic chronic rhinosinusitis (ECRS), making the distinction between the two very difficult. In this study, we evaluated the clinical findings of EGPA to identify its characteristics. Thirteen patients with EGPA who were referred to the department of otorhinolaryngology-head and neck surgery of Osaka University Hospital and 14 patients with ECRS were reviewed retrospectively. We reviewed the findings of blood tests (eosinophil count, MPO-ANCA/PR3-ANCA, antinuclear antibody, rheumatoid factor, IgE, IgG4), nasal symptoms, nasal polyps, computed tomography (CT) images of paranasal sinuses (Lund-Mackay score; LMS) and systemic symptoms. Results showed that patients with EGPA had significantly high eosinophil counts and IgE and IgG4 scores. In addition, some of them were positive for antinuclear antibodies and rheumatoid factors. Nasal symptoms, nasal polyps, and LMS were significantly less in patients with EGPA. Systemic symptoms in patients with EGPA, peripheral neuropathy, and lung lesions were often seen. When ECRS accompanied by extremely high eosinophil counts is encountered, we should be cautious about the development of EGPA and pay attention not only to ANCA but also to other autoantibodies, blood test results, and systemic symptoms.</p>