少年儿童抗中性粒细胞胞浆抗体相关原发性系统性血管炎的临床和病理特点

Clinical and pathological features of anti-neutrophil cytoplasm antibody associated systemic vasculitis in children

目的 回顾性总结并分析儿童抗中性粒细胞胞浆抗体 (ANCA)相关原发性系统性血管炎 (AASV)患者的临床和病理特点 ,并与成人原发AASV患者作比较 ,以提高对该类疾病的认识。方法 将原发AASV的儿童患者与同期成人原发AASV患者的临床和病理进行比较。结果 儿童组 2 0例 ,占同期诊断的原发AASV患者的 7 87% (2 0 / 2 5 4 ) ,与 38例成人住院患者相比 ,儿童组中女性患者比例显著高于成人组 (儿童组 80 % ;成人组 5 0 % ,P =0 0 4 7) ,二组在疾病组成和ANCA类型方面差异无显著性 ,均以显微镜下型多血管炎及P ANCA/抗MPO抗体阳性为主。儿童组高血压出现者显著少于成人组 (儿童组 2 0 % ;成人组 6 1% ,P =0 0 0 5 ) ,二组在肾脏受累 ,肾外器官受累 ,实验室检查 ,肾脏活检病理表现 ,治疗方案 ,临床缓解率方面均无显著性差异。结论 儿童原发AASV患者并不罕见 ,其临床与病理特点与成人原发AASV基本一致 。

Objective Anti-neutrophil cytoplasmic antibodies (ANCA) are serological diagnostic markers for certain small vessel vasculitises including Wegener′s granulomatosis and microscopic polyangiitis, which are also termed ANCA associated systemic vasculitis (AASV). The majority of patients with primary AASV reported are adults and predominantly elderly. Data on pediatric patients with primary AASV in China are lacking. This study aimed to analyze the clinical and pathological features of primary AASV in children.Methods Patients with primary AASV, admitted to the hospital within the past 7 years, were retrospectively studied. The clinical and pathological features were compared between pediatric and adult patients. In pediatric group, there were 20 cases with an average age of(12.1±4.1) years(aged from 5 to 17 years); in adult group, there were 38 cases with an average age of (55.3±14.1) years (aged from 20 to 78 years).Results The data of this study showed that pediatric patients accounted for 7.87% (20/254) of the whole primary AASV patients. Compared with 38 adult hospitalized patients, pediatric patients were predominantly female (80% vs 50%,P=0.047). Patients from both groups were microscopic polyangiitis predominantly (95% vs 74%, P>0.05) and the majority of the sera were P-ANCA/anti-MPO antibody positive in both groups (95% vs 74%, P>0.05). The prevalence of hypertension in pediatric patients was significantly lower than that in adults (20% vs 61%,P=0.005). There was no significant difference in clinical manifestations and clinical remission rates between the two groups.Conclusion Pediatric patients with AASV were not rare in China. The clinical and pathological features of patients with AASV in childhood were similar to adult patients, but there was a female predominance in children.