Department of Nephrology, Keiyu Hospital, 3-7-3 Minatomirai, Nishi-Ku, Yokohama, Kanagawa, 220-8521, Japan.
CEN Case Rep. 2023 Nov;12(4):368-373. doi: 10.1007/s13730-023-00778-7. Epub 2023 Feb 21.
Kimura disease (eosinophilic granuloma of the soft tissue) is a benign granulomatous disease complicated by nephrotic syndrome. Herein, we report a case of recurrent minimal change nephrotic syndrome (MCNS) complicated by Kimura disease that was successfully treated with rituximab. A 57-year-old man presented to our hospital with relapsed nephrotic syndrome with worsening swelling of the right anterior ear and elevated serum IgE. MCNS was diagnosed on renal biopsy. Treatment with 50 mg of prednisolone rapidly placed the patient in remission. Therefore, RTX 375 mg/m was added to the treatment regimen, and steroid therapy was tapered. Early steroid tapering was successful, and the patient is currently in remission. In this case, the nephrotic syndrome flare-up was accompanied by worsening Kimura disease. Rituximab reduced the worsening of symptoms related to Kimura disease, including head and neck lymphadenopathy and elevated IgE levels. Kimura disease and MCNS may share a common IgE-mediated type I allergic condition. Rituximab effectively treats these conditions. In addition, rituximab suppresses Kimura disease activity in patients with MCNS, enables early tapering of steroids, and reduces the total dose of steroids.
木村病（软组织嗜酸性肉芽肿）是一种良性肉芽肿性疾病，常并发肾病综合征。本文报告 1 例木村病并发复发性微小病变肾病综合征（MCNS）患者，经利妥昔单抗治疗后获得成功。1 例 57 岁男性因复发性肾病综合征就诊，右前耳肿胀加重，血清 IgE 升高。肾活检诊断为 MCNS。50mg 泼尼松治疗迅速缓解。因此，在治疗方案中加入 375mg/m RTX，逐渐减少激素用量。早期激素减量成功，患者目前处于缓解状态。在该病例中，肾病综合征复发伴木村病加重。利妥昔单抗减轻了与木村病相关的症状恶化，包括头颈部淋巴结病和 IgE 水平升高。木村病和 MCNS 可能具有共同的 IgE 介导的 I 型过敏状态。利妥昔单抗有效治疗这些疾病。此外，利妥昔单抗抑制 MCNS 患者的木村病活动，使激素早期减量，并减少激素总剂量。
