Gomes Lídia, Santos Sandra D, Pereira Samuel, Rua João, Fortuna Jorge
Pulmonology, University Hospital Center of Coimbra, Coimbra, PRT.
Internal Medicine, University Hospital Center of Coimbra, Coimbra, PRT.
Cureus. 2023 Feb 27;15(2):e35512. doi: 10.7759/cureus.35512. eCollection 2023 Feb.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic disease that develops with necrotizing granulomatous inflammation and is characterized by eosinophilia, asthma, and small vessel vasculitis. We report the case of a 74-year-old woman with a history of asthma, admitted to the Emergency Room with fever, headache, general malaise, weight loss and night sweats with one-month evolution, previously medicated with antibiotics without improvement. She presented with sinus palpation tenderness and lower leg bilateral sensitivity impairment. Laboratory tests showed neutrophilia and eosinophilia, normocytic anemia and elevated erythrocyte sedimentation rate and C-reactive protein. A computed tomography revealed sphenoid and maxillary sinusitis. Blood cultures and lumbar puncture were innocent. An extended autoimmune panel exposed a strong positive perinuclear anti-neutrophil cytoplasmic antibody - myeloperoxidase (pANCA-MPO). Sinus biopsy showed tissue infiltration by eosinophils, confirming EGPA. Corticosteroid (1 mg/kg/day) treatment was started with gradual improvement. Six months later there were no signs of active disease under prednisolone 10 mg and azathioprine 50 mg/day. This case highlights that refractory sinusitis in the presence of constitutional syndrome and peripheral eosinophilia should alert clinicians to the possibility of EGPA, particularly in patients with late-onset asthma.
嗜酸性肉芽肿性多血管炎(EGPA)是一种罕见的全身性疾病,伴有坏死性肉芽肿性炎症,其特征为嗜酸性粒细胞增多、哮喘和小血管血管炎。我们报告一例74岁有哮喘病史的女性病例,因发热、头痛、全身乏力、体重减轻和盗汗1个月进展情况而入住急诊室,此前使用抗生素治疗无改善。她出现鼻窦触压痛和双侧小腿感觉障碍。实验室检查显示中性粒细胞增多和嗜酸性粒细胞增多、正细胞性贫血以及红细胞沉降率和C反应蛋白升高。计算机断层扫描显示蝶窦和上颌窦炎。血培养和腰椎穿刺结果均为阴性。扩展的自身免疫检查显示核周抗中性粒细胞胞浆抗体-髓过氧化物酶(pANCA-MPO)强阳性。鼻窦活检显示嗜酸性粒细胞浸润组织,确诊为EGPA。开始使用皮质类固醇(1mg/kg/天)治疗,病情逐渐改善。6个月后,在泼尼松龙10mg和硫唑嘌呤50mg/天治疗下无活动性疾病迹象。该病例强调,在存在全身症状和外周嗜酸性粒细胞增多的情况下,难治性鼻窦炎应提醒临床医生注意EGPA的可能性,尤其是在迟发性哮喘患者中。