分析嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis, EGPA)的临床表现、诊断策略和治疗方案，提高对EGPA的早期识别和诊断能力。探讨单用美泊利珠单抗(mepolizumab)在抗中性粒细胞胞质抗体(antineutrophil cytoplasmic antibodies, ANCA)阴性EGPA患者中的疗效。

选择2024年7月26日我科收治的一例抗中性粒细胞胞质抗体阴性嗜酸性肉芽肿性多血管炎患者为对象，分析症状、体征、实验室检查、影像学检查及诊疗过程，并进行文献复习。

患者女性，43岁，因反复喘息、气促30余年，发现肺部阴影1月余入院。既往支气管哮喘、慢性鼻窦炎、鼻息肉病史。辅助检查示外周血白细胞计数8.74×10⁹/L，嗜酸性粒细胞(eosinophil, Eos)绝对计数0.90×10⁹/L，红细胞沉降率(erythrocyte sedimentation rate, ESR)31 mm/1 h，肺泡灌洗液(bronchoalveolar lavage fluid, BALF)中嗜酸性粒细胞比例升高至76%，总胆固醇6.70 mmol/L，低密度脂蛋白胆固醇3.98 mmol/L，脂蛋白A 344.0 mg/L，甘油三酯2.64 mmol/L。IgM阳性(1.00 COI)，IgG显著升高(>300.00 AU/ml)。抗中性粒细胞胞质抗体检测阴性。右肺中叶病理活检提示肺组织内嗜酸性粒细胞浸润。考虑诊断抗中性粒细胞胞质抗体阴性嗜酸性肉芽肿性多血管炎，单用美泊利珠单抗维持治疗，门诊随访复查血常规示嗜酸性粒细胞计数下降，胸部CT显示肺部病灶明显吸收。

长期支气管哮喘病史且外周血嗜酸性粒细胞持续升高的患者需警惕嗜酸性肉芽肿性多血管炎可能性，美泊利珠单抗可作为抗中性粒细胞胞质抗体阴性嗜酸性肉芽肿性多血管炎的有效维持治疗药物。

To analyze the clinical manifestations, diagnostic strategies, and treatment options for eosinophilic granulomatosis with polyangiitis (EGPA), and to improve the early recognition and diagnostic capability of eosinophilic granulomatosis with polyangiitis. Additionally, to explore the efficacy of Mepolizumab monotherapy in patients with antineutrophil cytoplasmic antibody (ANCA)-negative eosinophilic granulomatosis with polyangiitis.

A case of antineutrophil cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis admitted to our department on July 26, 2024, was selected. The symptoms, signs, laboratory tests, imaging findings, and treatment process were analyzed, and a literature review was conducted.

The patient was a 43-year-old female admitted due to recurrent wheezing and shortness of breath for over 30 years, along with the discovery of a pulmonary shadow for more than one month. She had a medical history of bronchial asthma, chronic sinusitis, and nasal polyps. Auxiliary examinations showed a peripheral white blood cell count of 8.74×10⁹/L, an absolute eosinophil count of 0.90×10⁹/L, an erythrocyte sedimentation rate of 31 mm/1h, and an elevated eosinophil proportion in bronchoalveolar lavage fluid to 76%. Total cholesterol 6.70 mmol/L, LDL cholesterol 3.98 mmol/L, Lipoprotein (a) 344.0 mg/L, Triglycerides 2.64 mmol/L. IgM positive (1.00 COI), IgG significantly elevated (>300.00 AU/ml). Antineutrophil cytoplasmic antibody testing was negative. Pathological biopsy of the right middle lung lobe revealed eosinophil infiltration in the lung tissue. A diagnosis of antineutrophil cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis was considered. The patient was treated with Mepolizumab monotherapy for maintenance. Follow-up outpatient examinations showed a decrease in eosinophil count, and chest CT revealed significant absorption of the pulmonary lesions.

Patients with a long-term history of asthma and persistently elevated peripheral blood eosinophil levels should be alert to the possibility of eosinophilic granulomatosis with polyangiitis. Mepolizumab can serve as an effective maintenance treatment for antineutrophil cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis.