Nephrology and Dialysis Center, Oswaldo Cruz German Hospital, São Paulo, Brazil.
Nephrology Division, University of São Paulo School of Medicine, São Paulo, Brazil.
Front Immunol. 2024 Jul 12;15:1404954. doi: 10.3389/fimmu.2024.1404954. eCollection 2024.
Kimura's disease (KD) is a rare chronic inflammatory disorder characterized by subcutaneous lymphoid hyperplasia with peripheral eosinophilia. Kidney involvement is reported in 15%-18% of adult patients with KD, in many cases as nephrotic syndrome. We present a case of overlapping membranous nephropathy and IgA nephropathy associated with KD.
A 27-year-old man was admitted with a history of bilateral leg edema for the last 2 months and concomitant progressive increase of cervical mass and fever. Laboratory findings were as follows: peripheral leukocyte count, 10,080/mm³; eosinophils, 3,200/mm³ (31.7%); serum creatinine, 0.83 mg/dL; and eGFR: 140 mL/min per 1.73 m. Urinalysis revealed the presence of hematuria and proteinuria and the following results: 24-h proteinuria, 12.9 g; serum albumin, 1.3 g/dL; and elevated IgE level, 750 kU/L. Serologies for hepatitis B, hepatitis C, HIV, and VDRL were all negative. Complement C3 and C4 levels were normal. No monoclonal protein was detected in blood and urine. Parasite infestation was discarded. A biopsy of the cervical lymph node revealed eosinophilic lymphoid hyperplasia, suggesting KD. A kidney biopsy revealed findings consistent with the overlapping of membranous nephropathy with IgA nephropathy. The patient was treated for KD with prednisone 1 mg/kg/d with progressive dose tapering and posterior association of methotrexate 15 mg/week. A renin-angiotensin system inhibitor was prescribed for nephrotic syndrome. The cervical mass regressed, and proteinuria achieved partial remission, with an increase in serum albumin level and normalization of eosinophils and IgE levels.
Although uncommon, kidney involvement must be considered in patients with KD. Glomerular diseases are the most frequent form of kidney injury.
木村病(KD)是一种罕见的慢性炎症性疾病,其特征为皮下淋巴组织增生伴外周血嗜酸性粒细胞增多。在 15%-18%的 KD 成年患者中可出现肾脏受累,在许多情况下表现为肾病综合征。我们报告了一例与 KD 相关的膜性肾病和 IgA 肾病重叠病例。
一名 27 岁男性,因双侧腿部水肿 2 个月,同时伴颈淋巴结肿大和发热逐渐加重而入院。实验室检查结果如下:外周白细胞计数 10080/mm³;嗜酸性粒细胞 3200/mm³(31.7%);血清肌酐 0.83 mg/dL;eGFR:140 mL/min/1.73 m²。尿分析显示血尿和蛋白尿,结果如下:24 小时蛋白尿 12.9 g;血清白蛋白 1.3 g/dL;IgE 水平升高至 750 kU/L。乙型肝炎、丙型肝炎、HIV 和 VDRL 血清学检查均为阴性。补体 C3 和 C4 水平正常。血和尿中均未检测到单克隆蛋白。寄生虫感染被排除。颈淋巴结活检显示嗜酸性淋巴组织增生,提示 KD。肾脏活检显示膜性肾病与 IgA 肾病重叠的表现。患者接受泼尼松 1 mg/kg/d 治疗 KD,随后逐渐减量,并联合每周 15 mg 甲氨蝶呤治疗。给予肾素-血管紧张素系统抑制剂治疗肾病综合征。颈淋巴结肿大消退,蛋白尿部分缓解,血清白蛋白水平升高,嗜酸性粒细胞和 IgE 水平恢复正常。
尽管罕见,但 KD 患者必须考虑肾脏受累。肾小球疾病是最常见的肾脏损伤形式。