Tanaka H, Waga S, Kakizaki Y, Sugimoto K, Ito T, Tateyama T
Department of Pediatrics, Hirosaki University, School of Medicine, Aomori, Japan.
Nihon Jinzo Gakkai Shi. 1996 Oct;38(10):463-8.
We report here a 15-year-old girl with myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA)-associated crescentic glomerulonephritis (CreGN) and subclinical autoimmune thyroiditis. She was found to have proteinuria and hematuria by a school mass-screening a year before the first visit to the hospital, where a routine examination revealed blood urea nitrogen (BUN) 36.8 mg/dl and serum creatinine concentration of 1.63 mg/dl, although she had no apparent disabilities. On admission, the additional laboratory findings showed proteinuria of 1.06 g/day, hematuria of 3+, and a creatinine clearance of 30.1 ml/min. Hypocomplementemia was not observed. A renal biopsy revealed pauci-immune CreGN with 95% fibrocellular crescents, 84% sclerosis and/or hyalinosis and a massive cellular infiltration in the interstitium. She had MPO-ANCA of 865 EU/ml and an anti-thyroid microsome antibody titer of 1:1,600 without the detection of anti-glomerular basement membrane antibodies. Laboratory tests and scintigraphies for the thyroid gland did not show any abnormalities. Under the diagnosis of MPO-ANCA-associated CreGN, cocktail therapy consisting of prednisolone, cyclophosphamide, dilazep hydrochloride and warfarin was started. Improvement of urinary abnormalities and suppression of further deterioration of renal function were observed. Serial renal biopsy 6 months after the initiation of therapy showed decrement of interstitial cell infiltration and no generation of other crescentic glomeruli. The patient's serum titer of anti-thyroid microsome antibody was not affected by the adsorption of MPO-reacted IgG, suggesting that MPO-ANCA was not cross-reactive to thyroid microsome antigen.
我们在此报告一名15岁女孩,患有髓过氧化物酶抗中性粒细胞胞浆抗体(MPO-ANCA)相关性新月体性肾小球肾炎(CreGN)和亚临床自身免疫性甲状腺炎。在首次就诊于医院前一年,她通过学校大规模筛查发现蛋白尿和血尿,尽管她没有明显不适,但常规检查显示血尿素氮(BUN)为36.8mg/dl,血清肌酐浓度为1.63mg/dl。入院时,其他实验室检查结果显示蛋白尿为1.06g/天,血尿为3+,肌酐清除率为30.1ml/min。未观察到补体降低。肾活检显示寡免疫性CreGN,有95%的纤维细胞性新月体,84%的硬化和/或玻璃样变性,间质有大量细胞浸润。她的MPO-ANCA为865EU/ml,抗甲状腺微粒体抗体滴度为1:1600,未检测到抗肾小球基底膜抗体。甲状腺的实验室检查和闪烁扫描未显示任何异常。在诊断为MPO-ANCA相关性CreGN后,开始使用泼尼松龙、环磷酰胺、盐酸地拉齐普和华法林组成的联合疗法。观察到尿液异常有所改善,肾功能进一步恶化得到抑制。治疗开始6个月后的系列肾活检显示间质细胞浸润减少,未出现其他新月体性肾小球。患者抗甲状腺微粒体抗体的血清滴度不受MPO反应性IgG吸附的影响,提示MPO-ANCA与甲状腺微粒体抗原无交叉反应。