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肾髓质血管炎伴皮肤白细胞碎裂性血管炎。

Renal Medullary Angiitis Associated with Cutaneous Leukocytoclastic Vasculitis.

机构信息

Department of Nephrology, Kuwana City Medical Center, Japan.

Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan.

出版信息

Intern Med. 2024 Jun 15;63(12):1757-1763. doi: 10.2169/internalmedicine.2679-23. Epub 2023 Nov 6.

Abstract

Renal medullary angiitis is characterized by interstitial hemorrhaging in the medulla with neutrophil infiltration. An 81-year-old man presented with a fever, kidney dysfunction, and purpura of the legs, which was diagnosed as leukocytoclastic vasculitis. Proteinase 3 antineutrophil cytoplasmic antibodies were weakly positive. A kidney biopsy showed severe tubulointerstitial hemorrhaging with neutrophilic infiltration in the perivascular areas surrounding the vasa recta in the medulla without crescent formation in the glomeruli. An immunofluorescence analysis was negative, and electron microscopy revealed no immune-dense deposits, ruling out immunoglobulin A vasculitis. Intravenous methylprednisolone for three days and plasma exchange followed by oral prednisolone improved his general condition.

摘要

肾髓质血管炎的特征是髓质间质出血伴中性粒细胞浸润。一名 81 岁男性因发热、肾功能不全和腿部紫癜就诊,被诊断为白细胞碎裂性血管炎。蛋白酶 3 抗中性粒细胞胞质抗体弱阳性。肾活检显示严重的肾小管间质出血,血管周围区域有中性粒细胞浸润,肾小球无新月体形成。免疫荧光分析阴性,电镜检查未见免疫复合物沉积,排除免疫球蛋白 A 血管炎。静脉注射甲基强的松龙 3 天,随后行血浆置换和口服泼尼松龙治疗后,患者一般情况改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce48/11239257/eecebab4efb2/1349-7235-63-1757-g001.jpg

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