儿童ANCA相关原发性小血管炎--韦格纳肉芽肿的探讨

目的：了解儿童抗中性粒细胞胞浆抗体(ANCA)相关原发性小血管炎——韦格纳肉芽肿的临床、病理特点及发病机制。方法：对1例韦格纳肉芽肿患儿的临床、实验室资料、病理进行分析，并复习相关文献。结果：韦格纳肉芽肿患儿临床上有多脏器损害、血尿、蛋白尿、肾功能不全；贫血与肾功能损害不一致；肺内结节、空洞；血沉增快、C反应蛋白升高；ANCA阳性，为C-ANCA/PR；扁桃体活检可见肉芽肿样病变，经皮质激素、细胞毒药物治疗，临床表现完全恢复。结论：儿童韦格纳肉芽肿临床表现及实验室检查与成人相似，对可疑患儿应及早进行ANCA检测和相应检查，早期诊断，及时治疗。

ANCA Related Idiopathic Small Vessel Vasculitis in Children-Wegener''''s Granulomatosis

Objective: To study the clinical and pathological characteristics of ANCA related idiopathic small vessel vasculitis in children-Wegener's granulomatosis. Methods:Clinical and pathological data was collected from one child with Wegener's granulomatosis and further analyzed.Results: Clinically, the child with Wegener's granulomatosis had multi-system involvement, hematuria, proteinuria and damaged renal function. Anemia was not consistent with damage of renal function. Chest X-ray showed pulmonary nodule and cavities. Except acceserated ESR and CRP, the child also had positive ANCA(C- ANCA/PR_3). Amygdalae biopsy tissues showed vasculitis granuloma. Following immunosuppression therapy, the symptoms of the child were totally comeback. Conclusion: The clinical and pathological characteristics of Wegener's granulomatosis in children were similar to those in adults. For patients with suspected small vessel vasculitis, an ANCA test should be performed in order to make an early diagnosis and treatment started in time.