儿童ANCA相关性血管炎呼吸系统临床表现及影像学结果分析

Clinical and imaging manifestations of the respiratory system in children with ANCA-associated vasculitis

目的探讨儿童抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)呼吸系统的临床表现和相关检查结果。方法分析2014年6月至2019年6月南京大学医学院附属金陵医院儿科收治的25例儿童ANCA相关性血管炎呼吸系统的临床表现、实验室检查和影像学检查资料。结果入组AAV患儿发病时呼吸系统临床表现主要以咽后壁淋巴滤泡增生、肺部啰音和发热为主;经治疗,入组患儿白细胞、中性粒细胞、血沉、降钙素原、白介素-6、支原体IgM抗体、呼吸道合胞病毒IgM抗体和肺炎链球菌感染量等数值明显降低(P<0.05,P<0.01);补体C3数值明显升高(P<0.05);免疫球蛋白IgE和IgG数值明显降低(P<0.05,P<0.01);CD4T细胞数值明显升高(P<0.05),CD3和CD8T细胞数值明显降低(P<0.05,P<0.01)。胸部CT结果示:20例患儿出现肺部损伤,其中以两肺损伤为主影像学表现以斑片状和絮状高密度影为主,并伴有胸腔积液和淋巴结肿大。随访至今,25例AAV患儿出现1例死亡3例进入ESRD期。肾活检后1年和2年的肾脏存活率分别为93.33%和77.78%。1年和2年的累积存活率都为93.33%。结论呼吸系统的表现在儿童AAV的临床表现中出现较早,但缺乏特异性。肺部影像学呈多病灶表现,亦缺乏特异性。补体系统和细胞免疫参与了AAV的发病和病情发展。

Objective To investigate the clinical and imaging manifestations of the respiratory system in children with antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV).Methods The clinical and imaging manifestations of the respiratory system as well as laboratory data of 25 AAV children who were treated from June 2014 to June 2018 at Department of Paediatrics,Jinling Hospital,School of Medicine,Nanjing University were analyzed.Results The main clinical manifestations of the respiratory system in children with AAV were proliferation of lymphoid follicles on the posterior pharyngeal wall,rales in the lungs,and fever.After treatment,white blood cell count,neutrophil count,erythrocyte sedimentation rate,procalcitonin,interleukin-6,mycoplasma IgM antibody,respiratory syncytial virus IgM antibody,and the number of Streptococcus pneumoniae in urine were significantly decreased(P<0.05 or P<0.01),while complement C3,IgE,IgG,CD4+T cells,CD3+T cells,and CD8+T cells were significantly increased(P<0.05 or P<0.01).Chest CT showed that 20 cases had lung injuries,most of which were bilateral.The imaging findings included patchy and flocculent high density shadows,accompanied by pleural effusions and lymph node enlargement.Follow-up of the 25 AAV children showed that one died and three had entered ESRD stage.The 1-year and 2-year kidney survival rates after renal biopsy were 93.33%and 77.78%,respectively.The cumulative survival rates at 1 and 2 years were both 93.33%.Conclusion Respiratory system manifestations appear early in the clinical manifestations of AAV in children,but lack specificity.Lung imaging examination can show multiple lesions,which also lack specificity.Complements and cellular immunity are involved in the pathogenesis and development of AAV.