Zhu Lisa, Dahhan Tamara
Department of Medicine, University of California Los Angeles (UCLA) Medical Center/David Geffen School of Medicine, Los Angeles, USA.
Cureus. 2025 Apr 28;17(4):e83131. doi: 10.7759/cureus.83131. eCollection 2025 Apr.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a small vessel vasculitis that most commonly presents with peripheral eosinophilia, upper airway disease, and asthma, and can also cause cutaneous, cardiac, neurologic, musculoskeletal, gastrointestinal, and renal disease. This case describes a 57-year-old male with a history of chronic sinusitis and asthma who presented with bilateral orbital inflammation causing proptosis and diplopia. He was initially treated with antibiotics for orbital cellulitis without benefit, then high-dose prednisone with taper for idiopathic orbital inflammation, with resolution of ocular symptoms. He was diagnosed with EGPA after rheumatologic evaluation. Serum Immunoglobulin G4 (IgG4) level was significantly elevated, but his clinical presentation was more consistent with EGPA than IgG4-related disease (IgG4-RD). The patient significantly improved with the addition of mepolizumab and later methotrexate. This case details a rare manifestation of EGPA-bilateral orbital inflammation and highlights the potential overlapping features between EGPA and IgG4-RD.
嗜酸性肉芽肿性多血管炎（EGPA）是一种小血管血管炎，最常见的表现为外周血嗜酸性粒细胞增多、上呼吸道疾病和哮喘，还可导致皮肤、心脏、神经、肌肉骨骼、胃肠道和肾脏疾病。本病例描述了一名57岁男性，有慢性鼻窦炎和哮喘病史，出现双侧眼眶炎症，导致眼球突出和复视。他最初因眼眶蜂窝织炎接受抗生素治疗但无效，随后因特发性眼眶炎症接受大剂量泼尼松并逐渐减量治疗，眼部症状得以缓解。经风湿病学评估后，他被诊断为EGPA。血清免疫球蛋白G4（IgG4）水平显著升高，但他的临床表现更符合EGPA而非IgG4相关性疾病（IgG4-RD）。加用美泊利单抗及随后的甲氨蝶呤后，患者病情明显改善。本病例详细介绍了EGPA的一种罕见表现——双侧眼眶炎症，并强调了EGPA与IgG4-RD之间潜在的重叠特征。
