儿童结节多动脉炎（PAN）是一种少见的系统性血管炎，诊治延误可导致后遗症发生率及死亡率增高。该文报道以可逆性后部白质脑病综合征（PRES）起病的儿童结节性多动脉炎1例。患儿，女，10岁，因发现高血压、伴抽搐1个月入院。抽搐前有头痛、呕吐及视物模糊，抽搐表现为全身性发作。入院前半年发现颈部包块。查体血压增高，无中枢神经系统阳性体征。头颅影像学特点为后头部长T1，长T2及FLAIR高信号、DWI等信号，抽搐原因考虑PRES。包块病检提示结节性多动脉炎，无结核依据；超声检查提示右侧肾脏萎缩，肾发射型计算机断层扫描（ECT）提示右肾多发血管狭窄、无血流灌注，确诊为PAN。对于多系统受累的血管疾病，排除常见的血管炎如川崎病及过敏性紫癜后需考虑PAN的可能，尽早行组织病理活检或血管成像检查，争取早期诊断，及早治疗。
Abstract
Childhood polyarteritis nodosa (PAN) is a rare systemic vasculitis and the delayed diagnosis and treatment will cause high incidence of sequelae and high mortality. This article reports a girl with childhood PAN due to posterior reversible encephalopathy syndrome (PRES). The girl aged ten years was admitted to the hospital due to hypertension and convulsions for one month. She had complaints of headache, vomiting, and blurred vision before convulsions. At six months before admission, a mass was observed in the neck. The physical examination showed that she had hypertension and no abnormal fndings in the central nervous system. The brain magnetic resonance imaging manifested long T1 and T2 signals, high signal intensities on fluid-attenuated inversion recovery (FLAIR) images, and iso-signal intensity on diffusion-weighted imaging in the white matter of the left occipital lobe. Therefore, the cause of convulsions was considered as PRES. Mass biopsy suggested PAN and no fndings supported tuberculosis. The right kidney atrophy was observed by ultrasound examination. Emission computed tomography of the kidney showed multiple vascular stenosis and no blood perfusion in the right kidney, so PAN was confrmed. These fndings suggest that PAN should be considered in patients with vasculitis who had involvements of multiple systems after excluding common vasculitis, such as Kawasaki disease and Henoch-Schönlein purpura. Biopsy and angiography should be performed as early as possible for timely diagnosis and treatment.
关键词
结节性多动脉炎 /
高血压 /
可逆性后部白质脑病综合征 /
儿童
Key words
Polyarteritis nodosa /
Hypertension /
Posterior reversible encephalopathy syndrome /
Child
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