Nephrology and Dialysis Unit, San Giovanni Evangelista Hospital, Tivoli, Italy.
Rheumatology Consultant, ASL Roma 5, Tivoli, Italy.
G Ital Nefrol. 2022 Apr 21;39(2):2022-vol2.
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic vasculitis. Rapidly progressive glomerulonephritis (RPGN) is a rare complication of EGPA. We report a case of a 60-year-old man, who is also a skilled cyclist, who was hospitalized to investigate a symptomatology that had arisen over the previous months and worsened in the last few weeks, to the point of limiting normal everyday activities. The physical examination revealed the presence of livedo reticularis of the four limbs, purpura of the lower limbs, arthritis of the ankles, and low-grade fever; the patient showed intense asthenia, loss of appetite, retrosternal heartburn, and a scarcely pharmacologically controlled asthma. He also reported weight loss (about 5 kg in the last 6 months). Rapidly progressing renal failure was observed with hyper-eosinophilia (4.7 thousand/μL eosinophils, 44% of total leukocytes), pulmonary opacities on chest computed tomography (CT), and sinusitis on CT of the facial massif. The search for antibodies directed against neutrophil cytoplasm (ANCA) revealed a high level of pANCA (pANCA ++, ELISA anti-MPO 666 UI/ml), associated with an increment of inflammation indicators. The induction therapy was high-dosage intravenous glucorticoids and cyclophosphamide, to improve the short and long-term prognosis. After 7 months of treatment, the patient reported a considerable improvement of the symptoms, which at that point did not necessitate pharmacological interventions. The eosinophils value was 0 cells/mm³, the inflammation indexes were back to the norm, and the renal function appeared significantly improved.
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,是一种罕见的系统性血管炎。急进性肾小球肾炎(RPGN)是 EGPA 的罕见并发症。我们报告了一例 60 岁男性患者的病例,他也是一名熟练的自行车手,因过去几个月出现的症状和最近几周恶化而住院,以至于限制了正常的日常活动。体格检查显示四肢网状青斑、下肢紫癜、踝关节关节炎和低热;患者表现出强烈的乏力、食欲不振、胸骨后烧心和哮喘难以用药物控制。他还报告体重减轻(过去 6 个月约 5 公斤)。迅速进展的肾衰竭伴有嗜酸性粒细胞增多(4700/μL 嗜酸性粒细胞,占总白细胞的 44%)、胸部计算机断层扫描(CT)上的肺部混浊和面部肿块的 CT 上的鼻窦炎。针对中性粒细胞胞质的抗体(ANCA)的检测发现高水平的 pANCA(pANCA++,ELISA 抗 MPO 666 UI/ml),伴有炎症指标的增加。诱导治疗采用高剂量静脉内糖皮质激素和环磷酰胺,以改善短期和长期预后。经过 7 个月的治疗,患者报告症状有了显著改善,此时不需要药物干预。嗜酸性粒细胞值为 0 细胞/mm³,炎症指标恢复正常,肾功能明显改善。