慢性活动性EB病毒感染合并系统性血管炎1例并文献复习

Chronic Active Epstein-Barr Virus Infection Combined with Systemic Vasculitis:a Case Report and Literature Review

目的提高对以皮肤损害起病的儿童慢性活动性EB病毒感染(CAEBV)合并心血管系统损害的认识。方法对中山大学附属第一医院收治的1例CAEBV合并系统性血管炎患儿的临床资料进行总结和分析,并复习相关文献。结果1)患儿起病隐匿,以反复皮肤损害起病,2次病灶活检证实EB病毒感染相关的皮肤损害。2)患儿除皮肤损害外,无其他的临床表现,但实验室检查提示EBV DNA载量4.53×10^6 copies·L^-1 ,EBV VCA-IgG阳性,IgA 6.17 g·L^-1 、IgG 23.2 g·L^-1 、lgG4 3.3 g·L^-1 、lgE 55 860 U·mL^-1 均升高,红细胞沉降率 66 mm· h^-1 ,CD3^+ 72%、CD19^+ 24.9%、CD3^+/CD4 +比值55.4%、CD3^+/CD8^+比值11.4%、NK细胞2.5%均降低,符合 CAEBV 的特征。3)心脏彩色多普勒超声提示主动脉窦部明显增宽,左右冠状动脉增宽并血栓形成;全身大动脉CT提示主动脉窦部明显扩张,双侧肺动脉扩张,冠状动脉扩张,腹腔干和肠系膜上动脉主干远段稍扩张;心电图提示ST-T改变。结论CAEBV有时临床表现不典型,可以反复皮肤损害起病。对反复皮肤损害者应行病灶活检,并行EBV及心脏彩色多普勒超声、CT血管重建等影像学检查,早期、及时地发现合并心血管损害等严重并发症有助于改善临床预后。

Objective To improve the understanding of chronic active Epstein-Barr virus infection(CAEBV) complicated by cardiovascular damage in children with skin lesions as the first manifestation. Methods Clinical data of a child treated in the First Affiliated Hospital of Sun Yat-sen University for CAEBV and systemic vasculitis were summarized and analyzed.Furthermore,the related literature was reviewed. Results In this case,the onset of the illness was concealed.It was caused by repeated skin damage.Two lesion biopsies confirmed that the skin lesions were associated with EB virus infection.In addition to skin damage,this child had no other clinical manifestations.However,laboratory tests showed positive EBV VCA-IgG with EBV DNA load of 4.53×10^6 copies·L^-1 .Moreover,the levels of IgA(6.17 g·L^-1 ),IgG(23.2 g·L^-1 ),lgG4(3.3 g·L^-1 ) and lgE(55 860 U·mL^-1 ) increased but the erythrocyte sedimentation rate(66 mm·h^-1 ),CD3^+(72%),CD19^+(24.9%),CD3^+/CD4^+ ratio(55.4%),CD3^+/CD8^+ ratio(11.4%) and NK cells( 2.5%) decreased in this child.These findings conformed to the characteristics of CAEBV.Cardiac color Doppler echocardiography showed that the aortic sinus was significantly widened,the left and right coronary arteries were widened and the thrombosis was formed.Whole-body aortic CT suggested the ectasia of aortic sinus,bilateral pulmonary arteries and coronary artery with slight dilatation of distal celiac trunk and main trunk of superior mesenteric artery in the distal segment.The ECG indicated the ST-T changes. Conclusion CAEBV sometimes has atypical clinical manifestations and can cause repeated skin damage.Lesion biopsy,cardiac color Doppler ultrasound,CT revascularization and other imaging examinations should be performed to early and timely find cardiovascular damage and other serious complications and to improve clinical prognosis in patients with repeated skin lesions.