Chen Ying, Wang Jinquan, Xu Feng, Zeng Caihong, Liu Zhihong
Clin Nephrol. 2016 Jun;85(6):332-9. doi: 10.5414/CN108749.
Kimura's disease (KD) with renal involvement is a rare disease. Optimal treatments are still not well established. It is necessary to analyze clinicopathological features, treatment responses, and prognosis for improving KD diagnosis and treatment.
Clinicopathological data, treatment responses, and prognosis were collected and analyzed retrospectively.
The patients consisted of 27 males and 2 females, with an average age of 35.5 ± 15.1 (13 - 61) years. 27 exhibited proteinuria ranging from 0.730 to 14.1 g/24 h (5.98 ± 3.40 g/24 h). Hypertension, renal insufficiency (serum creatinine (Scr) > 1.24 mg/dL), and microhematuria occurred in 4 (13.8%), 11 (37.9%), and 13 (44.8%) cases, respectively. Light microscopy (LM) identified mesangium proliferation, minimal change, focal and segmental glomerulosclerosis (FSGS), membranous glomerulonephritis, membranoproliferative glomerulonephritis (MPGN), and acute tubular necrosis in 14, 8, 3, 2, 1, and 1 cases, respectively. All were treated with Tripterygium wilfordii (TW), prednisone, leflunomide (LEF), tacrolimus (FK506), myophenolate mofetil (MMF), or renin-angiotensin system blockers (RASI). 26 patients were followed up for 1.60 - 108.7 months (39.6 ± 28.7). After treatments, urinary red blood cells (RBC) decreased in all. The amount of 24-hour urinary protein (24-hUPE) decreased in 24 patients. 22 reached complete remission (CR), 4 partial remissions (PR). The patients who did not relapse were younger than those who relapsed.
KD with renal involvement occurs predominantly among 35 - 50 year old Chinese patients with male predilection. The most common features are proteinuria, hypertension, micro hematuria with minimal change, and mesangial proliferative glomerulonephritis. Most were responsive to treatment, but could relapse. Gender, age, and hypertension are associated with KD recurrence. The prognosis is good mostly.
累及肾脏的木村病（KD）是一种罕见疾病。最佳治疗方案仍未完全确立。分析其临床病理特征、治疗反应及预后对于改善KD的诊断和治疗很有必要。
回顾性收集并分析临床病理数据、治疗反应及预后情况。
患者包括27例男性和2例女性，平均年龄35.5±15.1（13 - 61）岁。27例患者蛋白尿范围为0.730至14.1 g/24小时（平均5.98±3.40 g/24小时）。4例（13.8%）出现高血压，11例（37.9%）出现肾功能不全（血清肌酐（Scr）>1.24 mg/dL），13例（44.8%）出现镜下血尿。光镜（LM）检查发现14例有系膜增生、8例有微小病变、3例有局灶节段性肾小球硬化（FSGS）、2例有膜性肾小球肾炎、1例有膜增生性肾小球肾炎（MPGN）、1例有急性肾小管坏死。所有患者均接受雷公藤多苷（TW）、泼尼松、来氟米特（LEF）、他克莫司（FK506）、霉酚酸酯（MMF）或肾素 - 血管紧张素系统阻滞剂（RASI）治疗。26例患者随访1.60 - 108.7个月（平均39.6±28.7）。治疗后，所有患者尿红细胞（RBC）均减少。24例患者24小时尿蛋白量（24-hUPE）减少。22例达到完全缓解（CR），4例部分缓解（PR）。未复发患者比复发患者年轻。
累及肾脏的KD主要发生在35 - 50岁的中国男性患者中。最常见的特征是蛋白尿、高血压、镜下血尿伴微小病变以及系膜增生性肾小球肾炎。大多数患者对治疗有反应，但可能复发。性别、年龄和高血压与KD复发有关。总体预后良好。
