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儿童显微镜下多血管炎中的中枢神经系统血管炎

Central nervous system vasculitis in pediatric microscopic polyangiitis.

作者信息

Bhadu Danveer, Kumar Puneet, Malhotra Kiran Preet, Sharma Aarti, Sharma Meha, Srivastava Durgesh

机构信息

King George's Medical University, Lucknow.

Dr. RML Institute of Medical Sciences, Lucknow, India.

出版信息

Acta Reumatol Port. 2016 Oct-Dec;41(4):372-375.

Abstract

Microscopic polyangiitis is a small vessel vasculitis, associated with myeloperoxidaseantineutrophil cytoplasmic antibody. It rarely occurs in children. Central nervous system involvement in pediatric microscopic polyangiitis is not a well known entity with perhaps only five cases till date. We hereby present a 14-year-old girl with arthralgia, seizure, leukocytoclastic vasculitis, interstitial lung disease secondary to recurrent pulmonary hemorrhage, pauci-immune glomerulonephritis and high titers of MPO-ANCA, hence diagnostic of microscopic polyangiitis. Magnetic resonance imaging of brain showed diffuse parenchymal involvement which resolved at six months with development of new foci of microhemorrhages in different stages of evolution, reminiscent of vasculitis.

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摘要

显微镜下多血管炎是一种小血管血管炎,与髓过氧化物酶抗中性粒细胞胞浆抗体相关。它在儿童中很少见。小儿显微镜下多血管炎累及中枢神经系统并非广为人知的情况,迄今为止可能仅有5例。我们在此报告一名14岁女孩,有关节痛、癫痫发作、白细胞破碎性血管炎、继发于反复肺出血的间质性肺病、寡免疫性肾小球肾炎以及高滴度的髓过氧化物酶抗中性粒细胞胞浆抗体,从而诊断为显微镜下多血管炎。脑部磁共振成像显示弥漫性实质受累,6个月时病变消退,同时出现处于不同演变阶段的新微出血灶,提示血管炎。

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