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巨细胞病毒相关性坏死性血管炎,类似过敏性紫癜。

Cytomegalovirus-related necrotising vasculitis mimicking Henoch-Schönlein syndrome.

机构信息

Department of Paediatric Rheumatology, G. Gaslini Institute, Genoa, Italy.

出版信息

Clin Exp Rheumatol. 2014 May-Jun;32(3 Suppl 82):S73-5. Epub 2014 May 16.

Abstract

Viral vasculitides have been previously reported in the literature, the role of infections in their pathogenesis ranging from direct cause to trigger event. Here we report the case of a 3-year-old immunocompetent girl who developed a systemic vasculitis leading to ileal perforation, mimicking a full blown picture of Henoch-Schönlein purpura. High dosage steroid treatment was started, with good response. The anatomopathological examination of the resected gastrointestinal tract showed features of necrotising vasculitis and cytomegalovirus (CMV)-related inclusion bodies in the endothelial cells, with direct correlation to vascular damage. The causative role of viral infection was revealed by the presence of CMV DNA in patient's blood and positive IgG titer against the virus. Steroid therapy was then tapered: the patient achieved clinical remission, which still persists after a six-months follow-up. Our report suggests that CMV vasculitis is probably more frequent than previously thought, even in immunocompetent patients, with a protean clinical presentation, mimicking other types of vasculitides.

摘要

病毒相关性血管炎在文献中有过报道,感染在其发病机制中的作用范围从直接病因到触发因素。在这里,我们报告了一例 3 岁免疫功能正常的女孩发生了系统性血管炎,导致回肠穿孔,表现为典型的过敏性紫癜。我们开始使用大剂量的类固醇进行治疗,取得了良好的疗效。切除胃肠道的解剖病理检查显示出坏死性血管炎的特征,以及血管内皮细胞中的巨细胞病毒(CMV)相关包涵体,与血管损伤直接相关。通过患者血液中存在 CMV DNA 以及针对该病毒的 IgG 滴度阳性,证实了病毒感染的致病作用。随后逐渐减少类固醇治疗剂量:患者达到了临床缓解,在六个月的随访后仍然存在。我们的报告表明,CMV 血管炎的发病率可能比以前认为的要高,即使在免疫功能正常的患者中,其临床表现也多种多样,类似于其他类型的血管炎。

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