Department of Nephrology, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, School of Medicine, University of Electronic Science and Technology of China, No.32, West Section 2, Ring 1 Road, Chengdu, 610072, Sichuan, China.
BMC Nephrol. 2018 Nov 8;19(1):316. doi: 10.1186/s12882-018-1123-y.
Kimura's disease (KD) is a rare chronic inflammatory disorder with a high incidence of renal involvement. In this report, we present a case study of KD-associated nephrotic syndrome combined with minimal change disease (MCD) and acute renal tubular injury. Meanwhile, the clinical and histopathological characteristics of 26 patients with KD presenting with renal involvement were retrospectively evaluated.
Here, we report a case study of a 59-year-old male patient with KD confirmed by a lymph node biopsy. He developed widespread edema and decreased urine output. A palpable swollen mobile and non-tender lymph node behind the left ear was observed upon admission. A renal biopsy revealed minimal-change lesions and acute renal tubular injury. The patient received hemodialysis because of the oliguria and renal insufficiency, and an initial dose of 40 mg/d methylprednisolone and then continued treatment with 40 mg/d prednisolone. He exhibited a good clinical response to the steroid after 6 weeks of treatment. Of the other 26 patients included in the review, 13 patients presented with mesangial proliferative glomerulonephritis, 4 with membranous nephropathy, 3 with MCD, 3 with focal segmental glomerulosclerosis, 2 with IgA nephropathy and 1 with acute tubular injury. With the exception of 2 patients who progressed to end-stage renal disease and received hemodialysis, the majority of patients responded well to treatment with corticosteroids alone.
MCD combined with acute renal tubular injury is rare in patients with KD presenting with renal involvement. Corticosteroids may be a beneficial treatment for renal injury in patients with KD.
木村病（KD）是一种罕见的慢性炎症性疾病，肾受累发生率较高。本报告介绍了一例 KD 相关性肾病综合征合并微小病变病（MCD）和急性肾小管损伤的病例研究。同时，回顾性评估了 26 例肾受累 KD 患者的临床和组织病理学特征。
本报告介绍了一例经淋巴结活检确诊为 KD 的 59 岁男性患者。他出现广泛水肿和尿量减少。入院时观察到左耳后可触及肿胀、移动性和非触痛的淋巴结。肾活检显示微小病变和急性肾小管损伤。由于少尿和肾功能不全，患者接受了血液透析，初始剂量为 40mg/d 甲基强的松龙，然后继续用 40mg/d 泼尼松治疗。在治疗 6 周后，他对类固醇有良好的临床反应。在纳入的 26 例患者中，13 例表现为系膜增生性肾小球肾炎，4 例表现为膜性肾病，3 例表现为 MCD，3 例表现为局灶节段性肾小球硬化症，2 例表现为 IgA 肾病，1 例表现为急性肾小管损伤。除 2 例进展为终末期肾病并接受血液透析的患者外，大多数患者对单独使用皮质类固醇的治疗反应良好。
KD 肾受累患者中 MCD 合并急性肾小管损伤罕见。皮质类固醇可能对 KD 患者的肾损伤有益。
