Abstract：
Sarcoidosis is characterized by noncaseating granulomatous inflammation in multiple organs. Renal involvement is rare, and granulomatous interstitial nephritis (GIN) is the predominant histological feature. Renal sarcoidosis (RS) is usually diagnosed by exclusion, combining clinical and histological findings, and often remains misdiagnosed. This retrospective study aimed to describe the characteristics and prognosis of patients with RS in China.
Eighteen patients with RS from a single center were enrolled, of whom 15 had biopsy-proven tubulointerstitial nephritis. Their clinicopathological features and renal outcomes were analyzed to understand this rare disease better.
Eighteen patients (14 male/4 female) were included in our study. The median estimated glomerular filtration rate (eGFR) was 30.36 (11.57, 60.14) mL/min/1.73 m2. In 15 patients undergoing a renal biopsy, GIN was the most common pathological phenotype (66.67%). Seventeen patients had follow-up records, with a median follow-up of 24.07 (8.82, 60.90) months. One month after treatment, median eGFR increased significantly from 30.36 (11.57, 60.14) mL/min/1.73 m2 to 58.53 (39.35, 80.65) mL/min/1.73 m2, and proteinuria decreased from 1.10 (0.69, 1.58) g/24 h to 0.68 (0.52, 1.05) g/24 h. No patients relapsed or developed end-stage renal disease.
RS is a rare but important cause of tubulointerstitial injury, with a favorable long-term prognosis if properly diagnosed and treated promptly.
Eighteen patients with RS from a single center were enrolled, of whom 15 had biopsy-proven tubulointerstitial nephritis. Their clinicopathological features and renal outcomes were analyzed to understand this rare disease better.
Eighteen patients (14 male/4 female) were included in our study. The median estimated glomerular filtration rate (eGFR) was 30.36 (11.57, 60.14) mL/min/1.73 m2. In 15 patients undergoing a renal biopsy, GIN was the most common pathological phenotype (66.67%). Seventeen patients had follow-up records, with a median follow-up of 24.07 (8.82, 60.90) months. One month after treatment, median eGFR increased significantly from 30.36 (11.57, 60.14) mL/min/1.73 m2 to 58.53 (39.35, 80.65) mL/min/1.73 m2, and proteinuria decreased from 1.10 (0.69, 1.58) g/24 h to 0.68 (0.52, 1.05) g/24 h. No patients relapsed or developed end-stage renal disease.
RS is a rare but important cause of tubulointerstitial injury, with a favorable long-term prognosis if properly diagnosed and treated promptly.
摘要：
背景：结节病的特征是多器官的非干酪性肉芽肿性炎症。肾脏受累是罕见的，肉芽肿性肾小管间质性肾炎（GIN）是主要的组织学特征。肾结节病（RS）通常通过排除来诊断。结合临床和组织学发现，并且经常被误诊。这项回顾性研究旨在描述中国RS患者的特征和预后。
方法：纳入来自一个中心的18例RS患者，其中15人患有活检证实的肾小管间质性肾炎。分析他们的临床病理特征和肾脏结局，以更好地了解这种罕见疾病。
结果：18例患者（14例男性/4例女性）纳入本研究。eGFR中位数为30.36（11.57，60.14）ml/min/1.73ml。在接受肾活检的15名患者中，GIN是最常见的病理表型(66.67%)。17例患者有随访记录,中位随访时间为24.07（8.82，60.90）个月。治疗后一个月，估计肾小球滤过率（eGFR）中位数从30.36（11.57，60.14）ml/min/1.73m2显着增加到58.53（39.35，80.65）ml/min/1.73m2，蛋白尿从1.10（0.69，1.58）g/24h降至0.68（0.52，1.05）g/24h。没有患者复发或发展为终末期肾病。
结论：RS是肾小管间质损伤的一个罕见但重要的原因，如果正确诊断和及时治疗，长期预后良好。
方法：纳入来自一个中心的18例RS患者，其中15人患有活检证实的肾小管间质性肾炎。分析他们的临床病理特征和肾脏结局，以更好地了解这种罕见疾病。
结果：18例患者（14例男性/4例女性）纳入本研究。eGFR中位数为30.36（11.57，60.14）ml/min/1.73ml。在接受肾活检的15名患者中，GIN是最常见的病理表型(66.67%)。17例患者有随访记录,中位随访时间为24.07（8.82，60.90）个月。治疗后一个月，估计肾小球滤过率（eGFR）中位数从30.36（11.57，60.14）ml/min/1.73m2显着增加到58.53（39.35，80.65）ml/min/1.73m2，蛋白尿从1.10（0.69，1.58）g/24h降至0.68（0.52，1.05）g/24h。没有患者复发或发展为终末期肾病。
结论：RS是肾小管间质损伤的一个罕见但重要的原因，如果正确诊断和及时治疗，长期预后良好。
