Department of Laboratory Medicine, The First Hospital of Hebei Medical University, Shijiazhuang, China.
Physical Examination Center, The First Hospital of Hebei Medical University, Shijiazhuang, China.
Ann Palliat Med. 2021 Oct;10(10):11209-11215. doi: 10.21037/apm-21-2236.
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is a rare chronic necrotizing eosinophilic granulomatous inflammatory disease characterized by eosinophil-rich granulomatous inflammation and small- to medium-size vessel vasculitis associated with bronchial asthma and eosinophilia, which is positive for anti-neutrophil cytoplasmic antibody (ANCA) in approximately 50-70% of cases. We report a case of a 23-year-old woman was admitted to our hospital because of a of small vesicles on both lower limbs and a 4-month history of small scattered skin rash with pruritus V6 on both lower limbs four-month history of scattered skin rash with pruritus. Laboratory data from peripheral blood revealed leukocytosis, eosinophilia, thrombocytosis, hyperfibrinolysis, and mild renal injury. Her ANCA was negative, and the skin pathological examination showed granuloma lesions with eosinophils, while elevated eosinophils were also found in the bone marrow. EGPA was diagnosed. On the other hand, the patient had 2-year-long rhinosinusitis, 9-month-long nephrotic syndrome, and 1-month-long dry cough, which might be a type of asthma. With steroid therapy followed by systemic immunomodulatory therapy, the patient's symptoms were relieved. Our case report and literature review highlight the importance of recognizing cough variant asthma as an initial presenting symptom of EGPA, providing an opportunity for early diagnosis and treatment to reduce the risk of further disease progression and morbidity.
嗜酸性肉芽肿性多血管炎(EGPA),以前称为变应性肉芽肿性血管炎,是一种罕见的慢性坏死性嗜酸性粒细胞性炎症性疾病,其特征为嗜酸性粒细胞丰富的肉芽肿性炎症和小至中等大小血管血管炎,伴有支气管哮喘和嗜酸性粒细胞增多症,约 50-70%的病例抗中性粒细胞胞质抗体(ANCA)阳性。我们报告了一例 23 岁女性,因双下肢小水疱和 4 个月下肢散在瘙痒性小皮疹史入院。实验室检查显示白细胞增多、嗜酸性粒细胞增多、血小板增多、纤维蛋白溶解亢进和轻度肾损伤。她的 ANCA 阴性,皮肤病理检查显示有嗜酸性粒细胞的肉芽肿病变,骨髓中也发现嗜酸性粒细胞升高。诊断为 EGPA。另一方面,患者有 2 年鼻-鼻窦炎、9 个月肾病综合征和 1 个月干咳,可能是哮喘的一种。经激素治疗和全身免疫调节治疗后,患者症状缓解。我们的病例报告和文献复习强调了认识咳嗽变异性哮喘作为 EGPA 的初始表现的重要性,为早期诊断和治疗提供了机会,可降低疾病进一步进展和发病的风险。