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目的 了解儿童原发性小血管炎致急进性肾炎 (RPGN)Ⅲ型的临床、病理特点及发病机制。方法 对 1例原发性小血管炎致RPGNⅢ型患儿的临床、实验室资料、病理进行分析 ,并复习相关文献。结果 此例RPGNⅢ型患儿临床上有血尿、蛋白尿、肾功能损害、贫血、血淀粉酶增高、抗中性粒细胞胞浆抗体 (ANCA)阳性 ,为 p ANCA ;血沉快 (94mm/h) ,血γ 球蛋白增高 ,肾脏病理改变为广泛新月体形成 ;肾小球广泛纤维化及变性、坏死 ,免疫荧光IgM弱阳性 ,IgA、IgG、C3、C1q、纤维蛋白 (Fib)均阴性。经皮质激素、细胞毒药物治疗 ,肾功能损害得到控制 ,临床表现改善。结论 儿童RPGNⅢ型可因原发性小血管炎引起 ,其临床表现、肾脏病理改变与成人相似 ,但有其特点。
Objective To investigate the clinical, pathological features and pathogenesis of children with primary small vessel vasculitis and acute progressive nephritis (RPGN) type Ⅲ. Methods A case of primary small vasculitis caused RPGN Ⅲ type of children clinical, laboratory data, pathological analysis, and review the relevant literature. Results The patients with RPGN Ⅲ type had clinically hematuria, proteinuria, renal dysfunction, anemia, elevated amylase, anti-neutrophil cytoplasmic antibody (ANCA) , Increased serum gamma globulin and extensive crescent formation in renal pathology; extensive glomerular fibrosis and degeneration, necrosis, immunofluorescence IgM weak positive, IgA, IgG, C3, C1q, fibrin (Fib) were negative. Corticosteroids, cytotoxic drugs, renal dysfunction was controlled, clinical manifestations improved. Conclusion RPGN type Ⅲ in children may be caused by primary vasculitis. The clinical manifestations and pathological changes of kidney are similar to those in adults, but have their own characteristics.