Çelakıl Mehtap Ezel, Yücel Burcu Bozkaya, Özod Umay Kiraz, Bek Kenan
Kocaeli University, School of Medicine, Department of Pediatric Nephrology and Pathology, Kocaeli, Turkey.
J Bras Nefrol. 2019 Apr-Jun;41(2):293-295. doi: 10.1590/2175-8239-jbn-2018-0062. Epub 2018 Sep 6.
Pauci-immune glomerulonephritis (GN) is more common in elderly people compared to children and the etiology is not completely understood yet. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. We report a case of a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci-immune crescentic glomerulonephritis with crescents in 20 of 25 glomeruli (80%). Of these 20 crescents, 12 were cellular, 4 fibrocellular, and 4 globally sclerotic. She did not have purpura, arthritis, or systemic symptoms and she responded well to initial immunosuppressive treatment despite relatively severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30 mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2 mg/kg per day) orally for 3 months with prednisone (1 mg/kg per day). In one month, remission was achieved with normal serum creatinine and prednisone was gradually tapered. The case of this child with a relatively rare pediatric disease emphasizes the importance of early and aggressive immunosuppressive treatment in patients with renal-limited ANCA-associated pauci-immune crescentic GN even if with a mild clinical presentation. As in our patient, clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is mandatory in such children to guide the clinical management and predict prognosis.
与儿童相比，寡免疫性肾小球肾炎（GN）在老年人中更为常见，其病因尚未完全明确。80%的患者抗中性粒细胞胞浆抗体（ANCA）呈阳性。我们报告一例7岁女孩，她因全身不适和肌酐轻度升高就诊，被诊断为ANCA相关性寡免疫性新月体性肾小球肾炎，25个肾小球中有20个出现新月体（80%）。在这20个新月体中，12个为细胞性，4个为纤维细胞性，4个为全球硬化性。她没有紫癜、关节炎或全身症状，尽管组织病理学相对严重，但对初始免疫抑制治疗反应良好。患者最初接受了三次静脉注射甲泼尼龙冲击治疗（隔日30mg/kg），并继续口服环磷酰胺（CYC；每日2mg/kg）3个月，同时服用泼尼松（每日1mg/kg）。1个月内血清肌酐恢复正常，病情缓解，泼尼松逐渐减量。该患有相对罕见儿科疾病的儿童病例强调，即使临床表现较轻，对于肾脏局限性ANCA相关性寡免疫性新月体性GN患者，早期积极的免疫抑制治疗也很重要。正如我们的患者一样，临床和实验室检查结果可能并不总是能准确反映肾脏组织病理学的严重程度，因此对于此类儿童，进行肾活检以指导临床管理和预测预后是必不可少的。
