Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
Department of Thoracic Surgery, Seoul National University Hospital, Seoul, Korea.
Kidney Res Clin Pract. 2014 Sep;33(3):157-60. doi: 10.1016/j.krcp.2014.06.002. Epub 2014 Aug 23.
Kimura disease (KD) is an eosinophilic, granulomatous, benign, chronic inflammatory disease with an unknown etiology. A 33-year-old woman visited our hospital because of a palpable, left subclavian mass, a left scapulo-anterior pseudoaneurysm, and nephrotic syndrome. Her subclavian lymph node biopsy examination result was consistent with KD, and results of a renal biopsy indicated secondary membranous nephropathy. After renal histological examination confirmed nephropathy, treatment with prednisolone and cyclosporine was initiated, which was maintained for over 1 year. However, this therapy only provided a transient improvement in proteinuria. One year after commencing the treatment, both proteinuria and azotemia aggravated as the left axillary mass doubled in size. Finally, the mass was surgically excised, following which the azotemia rapidly normalized and proteinuria resolved within 1 month. This case shows that tumor resection in a patient with KD with secondary nephropathy may resolve secondary renal manifestations. Furthermore, reversible renal dysfunction may be caused by unknown secreted molecules.
木村病(KD)是一种原因不明的嗜酸性粒细胞性、肉芽肿性、良性、慢性炎症性疾病。一位 33 岁女性因触及性、左侧锁骨下肿块、左侧肩胛前假性动脉瘤和肾病综合征就诊于我院。其锁骨下淋巴结活检结果符合 KD,肾活检结果提示为继发性膜性肾病。肾组织学检查确认肾病后,开始使用泼尼松龙和环孢素治疗,持续了 1 年以上。然而,这种治疗仅使蛋白尿得到短暂改善。治疗开始后 1 年,蛋白尿和氮质血症加重,左侧腋窝肿块增大了 1 倍。最终,肿块被手术切除,氮质血症迅速恢复正常,1 个月内蛋白尿也得到了缓解。本病例表明,继发性肾病的 KD 患者行肿瘤切除术可能会使继发性肾脏表现得到缓解。此外,可能是由于未知的分泌分子导致肾功能可逆性障碍。