两例特发性类固醇抵抗型肾病综合征合并血栓性微血管病。
Two cases of idiopathic steroid-resistant nephrotic syndrome complicated with thrombotic microangiopathy.
机构信息
Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
Department of Pediatrics, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan.
出版信息
BMC Nephrol. 2020 Aug 3;21(1):323. doi: 10.1186/s12882-020-01985-5.
BACKGROUND
Thrombotic microangiopathy (TMA) is a histopathological entity associated with microangiopathic hemolytic anemia, thrombocytopenia, and end-organ ischemic damage. Although TMA is caused by various diseases, there have been few reports regarding children with idiopathic nephrotic syndrome (NS) and TMA. Here we report two 1-year-old infants with steroid-resistant NS (SRNS) who presented with severe hypertension, acute kidney injury (AKI), and TMA.
CASE PRESENTATION
The diagnosis of NS was complicated with anemia, AKI, and hypertension. Maximum blood pressure was 150/70 mmHg in Case 1 and 136/86 mmHg in Case 2. There was no thrombocytopenia during their clinical course in both cases. Renal biopsy showed the features of TMA, including endothelial cell swelling, capillarectasia or marked mesangiolysis, along with mesangial proliferation in Case 1 and TMA with minor glomerular abnormalities in Case 2. Hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, and secondary TMA other than that caused by hypertension were excluded. Oral prednisolone therapy, frequent infusion of albumin and diuretics, and multiple anti-hypertensive drugs were initiated. Blood pressure was controlled after 6 and 7 days from initiation of multiple anti-hypertensive drugs and lisinopril was added due to persistent mild proteinuria and mild hypertension after improvement of renal function in both cases. Proteinuria resolved completely 4 months after admission with daily oral prednisolone for 4 weeks followed by alternative daily oral prednisolone for 4 weeks in Case 1. Proteinuria resolved completely 10 months after admission with initial prednisolone treatment for 4 weeks followed by cyclosporine A and intravenous methylprednisolone pulse therapy in Case 2. The follow-up biopsy showed no TMA findings in both patients. Because the patient in Case 1 subsequently developed frequent relapsing NS, cyclosporine A was commenced after the second biopsy and he did not have any flares for 2 years. Renal function was normal in Case 1 and mildly decreased in Case 2 at last follow-up (creatinine-eGFR of 136.2 mL/min/cm in Case 1 and 79.5 mL/min/cm in Case 2).
CONCLUSION
Severe hypertension and AKI can be signs of TMA in patients with SRNS. Strict anti-hypertensive therapy might improve renal outcomes.
背景
血栓性微血管病（TMA）是一种与微血管性溶血性贫血、血小板减少和终末器官缺血性损伤相关的组织病理学实体。尽管 TMA 由各种疾病引起，但关于特发性肾病综合征（NS）和 TMA 的儿童病例报告较少。本文报道了 2 例 1 岁的类固醇耐药性 NS（SRNS）患儿，他们表现为严重高血压、急性肾损伤（AKI）和 TMA。
病例介绍
NS 合并贫血、AKI 和高血压，诊断复杂。1 例患儿最高血压为 150/70mmHg，另 1 例为 136/86mmHg。2 例患儿在整个病程中均无血小板减少。肾脏活检显示 TMA 的特征，包括内皮细胞肿胀、毛细血管扩张或明显系膜溶解，1 例伴有系膜增生，另 1 例伴有轻微肾小球异常。排除了溶血尿毒综合征、血栓性血小板减少性紫癜和高血压以外的继发性 TMA。患儿开始口服泼尼松龙治疗，频繁输注白蛋白和利尿剂，并使用多种降压药物。2 例患儿在开始使用多种降压药物后 6 和 7 天血压得到控制，并在肾功能改善后，因蛋白尿持续存在且轻度高血压，加用赖诺普利。1 例患儿在入院后 4 周内每天口服泼尼松龙治疗 4 周，然后隔日口服泼尼松龙治疗 4 周，4 个月后蛋白尿完全缓解。2 例患儿在入院后 4 周内接受初始泼尼松龙治疗，然后接受环孢素 A 和静脉甲基泼尼松龙脉冲治疗，10 个月后蛋白尿完全缓解。在 2 例患儿的随访活检中均未发现 TMA 表现。由于 1 例患儿随后出现频繁复发的 NS，在第 2 次活检后开始使用环孢素 A，2 年内未再出现活动。1 例患儿的肾功能正常，2 例患儿的肾功能轻度下降（1 例患儿的肌酐-eGFR 为 136.2mL/min/cm，2 例患儿的肌酐-eGFR 为 79.5mL/min/cm）。
结论
SRNS 患者严重高血压和 AKI 可能是 TMA 的表现。严格的降压治疗可能改善肾脏结局。
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