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1例结节性多动脉炎合并椎动脉血管炎经托珠单抗和环磷酰胺成功治疗

A Case of Polyarteritis Nodosa Associated with Vertebral Artery Vasculitis Treated Successfully with Tocilizumab and Cyclophosphamide.

作者信息

Watanabe Kae, Rajderkar Dhanashree A, Modica Renee F

机构信息

Department of Pediatrics, University of Florida, 1600 SW Archer Road, Gainesville, FL 32608, USA.

Department of Radiology, University of Florida, Gainesville, FL 32608, USA.

出版信息

Case Rep Pediatr. 2016;2016:7987081. doi: 10.1155/2016/7987081. Epub 2016 Jan 20.

Abstract

Pediatric polyarteritis nodosa is rare systemic necrotizing arteritis involving small- and medium-sized muscular arteries characterized by aneurysmal dilatations involving the vessel wall. Aneurysms associated with polyarteritis nodosa are common in visceral arteries; however intracranial aneurysms have also been reported and can be associated with central nervous system symptoms, significant morbidity, and mortality. To our knowledge extracranial involvement of the vertebral arteries has not been reported but has the potential to be deleterious due to fact that they supply the central nervous system vasculature. We present a case of a 3-year-old Haitian boy with polyarteritis nodosa that presented with extracranial vessel involvement of his vertebral arteries. After thorough diagnostic imaging, including a bone scan, ultrasound, Magnetic Resonance Imaging/Angiography, and Computed Tomography Angiography, he was noted to have vertebral artery vasculitis, periostitis, subacute epididymoorchitis, arthritis, and myositis. He met diagnostic criteria for polyarteritis nodosa and was treated with cyclophosphamide, methylprednisolone, and tocilizumab, which resulted in improvement of his inflammatory markers, radiographic findings, and physical symptoms after treatment. To the authors' knowledge, this is the first report of vertebral artery vasculitis in polyarteritis nodosa as well as successful treatment of the condition using the combination cyclophosphamide and tocilizumab for this condition.

摘要

小儿结节性多动脉炎是一种罕见的系统性坏死性动脉炎,累及中小肌性动脉,其特征为血管壁出现动脉瘤样扩张。与结节性多动脉炎相关的动脉瘤在内脏动脉中很常见;然而,颅内动脉瘤也有报道,可伴有中枢神经系统症状、严重的发病率和死亡率。据我们所知,椎动脉颅外受累尚未见报道,但由于椎动脉供应中枢神经系统血管,其受累可能有害。我们报告一例3岁海地男孩患结节性多动脉炎,出现椎动脉颅外血管受累。经过全面的诊断性影像学检查,包括骨扫描、超声、磁共振成像/血管造影和计算机断层血管造影,发现他患有椎动脉血管炎、骨膜炎、亚急性附睾睾丸炎、关节炎和肌炎。他符合结节性多动脉炎的诊断标准,接受了环磷酰胺、甲泼尼龙和托珠单抗治疗,治疗后炎症指标、影像学表现和身体症状均有所改善。据作者所知,这是结节性多动脉炎中椎动脉血管炎的首例报道,也是使用环磷酰胺和托珠单抗联合治疗该病取得成功的首例报道。

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