Kanno Shuto, Kawasaki Yukihiko, Maeda Ryo, Miyazaki Kyohei, Ono Atsushi, Suzuki Yuichi, Suyama Kazuhide, Suzuki Shigeo, Hosoya Mitsuaki
Department of Pediatrics, Fukushima Medical University School of Medicine, 1 Hikariga-oka, Fukushima City, Fukushima, 960-1295, Japan.
CEN Case Rep. 2014 Nov;3(2):232-236. doi: 10.1007/s13730-014-0126-1. Epub 2014 May 27.
Antineutrophil cytoplasmic autoantibody-associated glomerulonephritis (GN) in childhood is rare and has a poor prognosis. We report an 11-year-old girl with renal-limited antineutrophil cytoplasmic autoantibody-associated vasculitis. Proteinuria and hematuria were first detected by a school urinary screening (SUS) program. Histopathological examination revealed pauci-immune necrotizing GN. She did not display purpura or peripheral neuropathy. She was diagnosed with antineutrophil cytoplasmic autoantibody-associated GN based on proteinuria, high serum titers of antineutrophil cytoplasmic autoantibodies (ANCAs), and pauci-immune necrotizing GN. The patient was treated with combination therapy, consisting of methylprednisolone and urokinase pulse, prednisolone, mizoribine (MZB), warfarin, and dilazep hydrochloride. At 2 months after treatment, urinary protein excretion was decreased and the hematuria had disappeared, while the serum titer of ANCAs was also decreased. The dose of prednisolone was tapered, and proteinuria and hematuria later disappeared at 9 months after treatment. In conclusion, we reported an 11-year-old girl with renal-limited antineutrophil cytoplasmic autoantibody-associated vasculitis early identified by a SUS program and treated with multi-drug combination therapy including MZB. On the basis of our results, we believe that a SUS programs may be effective for the early identification and treatment of children with renal-limited antineutrophil cytoplasmic autoantibody-associated vasculitis.
儿童抗中性粒细胞胞浆自身抗体相关性肾小球肾炎（GN）较为罕见，预后较差。我们报告一名11岁患有限局于肾脏的抗中性粒细胞胞浆自身抗体相关性血管炎的女孩。蛋白尿和血尿最初是通过学校尿液筛查（SUS）项目检测到的。组织病理学检查显示寡免疫坏死性GN。她未出现紫癜或周围神经病变。基于蛋白尿、高血清抗中性粒细胞胞浆自身抗体（ANCA）滴度以及寡免疫坏死性GN，她被诊断为抗中性粒细胞胞浆自身抗体相关性GN。患者接受了联合治疗，包括甲泼尼龙和尿激酶冲击治疗、泼尼松龙、咪唑立宾（MZB）、华法林和盐酸地拉齐普。治疗2个月后，尿蛋白排泄减少，血尿消失，同时ANCA血清滴度也降低。泼尼松龙剂量逐渐减少，治疗9个月后蛋白尿和血尿最终消失。总之，我们报告了一名11岁患有限局于肾脏的抗中性粒细胞胞浆自身抗体相关性血管炎的女孩，通过SUS项目早期确诊，并接受了包括MZB在内的多药联合治疗。基于我们的结果，我们认为SUS项目可能对早期识别和治疗患有限局于肾脏的抗中性粒细胞胞浆自身抗体相关性血管炎的儿童有效。
