Renal Division, Department of Medicine, Peking University First Hospital, Beijing, People's Republic of China.
Institute of Nephrology, Peking University, Beijing, People's Republic of China.
Clin J Am Soc Nephrol. 2022 Apr;17(4):527-534. doi: 10.2215/CJN.12890921. Epub 2022 Feb 24.
Patients with monoclonal gammopathy and concomitant kidney diseases are frequently found in clinical practice. Some of them are diagnosed with monoclonal gammopathy of renal significance (MGRS) due to the presence of monoclonal Ig-related kidney injuries. This study aimed to investigate the histopathologic spectrum and clinical characteristics associated with MGRS in a large cohort of patients with monoclonal gammopathy and biopsy-proven kidney diseases from a single Chinese nephrology referral center.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Patients who presented with monoclonal gammopathy (monoclonal spike on serum and/or urine immunofixation tests) and underwent kidney biopsy in the Peking University First Hospital from January 1, 1999 to December 31, 2020 were enrolled in this retrospective study. Patients with malignant hematologic diseases were excluded. Clinical and laboratory data were collected from the electronic medical record system. Comparisons of patients with and without MGRS and with and without amyloidosis were performed. The clinical characteristics associated with MGRS were identified using multivariable logistic regression.
A total of 700 patients with monoclonal gammopathy and kidney biopsy were identified. Thirteen patients with repeat kidney biopsies were analyzed separately. For the remaining 687 patients with one kidney biopsy, 261 patients (38%) had MGRS lesions, and the rest (426 patients, 62%) had non-MGRS kidney diseases. Ig-related amyloidosis accounted for the most MGRS cases (=164, 63%), followed by monoclonal Ig deposition disease (=23, 9%) and thrombotic microangiopathy (=22, 8%). In the non-MGRS group, membranous nephropathy was the most common diagnosis (=171, 40%). In the multivariable logistic regression model, the presence of abnormal serum free light chain ratio, older age, and greater proteinuria were independently associated with MGRS.
Monoclonal Ig amyloidosis is the leading cause of MGRS in Chinese patients with monoclonal gammopathy. The presence of abnormal free light chain ratio, older age, and greater proteinuria were associated with MGRS.
在临床实践中经常会发现患有单克隆丙种球蛋白病和伴发肾脏疾病的患者。其中一些患者由于存在单克隆免疫球蛋白相关的肾脏损伤而被诊断为单克隆丙种球蛋白相关性肾损害(MGRS)。本研究旨在调查来自中国一家肾病转诊中心的一组患有单克隆丙种球蛋白病和经肾活检证实的肾脏疾病的患者中与 MGRS 相关的组织病理学谱和临床特征。
设计、地点、参与者和测量方法:本回顾性研究纳入了 1999 年 1 月 1 日至 2020 年 12 月 31 日期间在北京大学第一医院就诊的患有单克隆丙种球蛋白病(血清和/或尿液免疫固定试验中出现单克隆峰)并接受肾活检的患者。排除患有恶性血液病的患者。从电子病历系统中收集临床和实验室数据。比较了有和无 MGRS 以及有和无淀粉样变性的患者。使用多变量逻辑回归确定与 MGRS 相关的临床特征。
共纳入 700 例患有单克隆丙种球蛋白病和肾活检的患者。对另外 13 例接受重复肾活检的患者进行了单独分析。对于其余 687 例接受单次肾活检的患者,261 例(38%)患有 MGRS 病变,其余 426 例(62%)患有非 MGRS 肾脏疾病。Ig 相关性淀粉样变性占 MGRS 病例最多(=164,63%),其次是单克隆 Ig 沉积病(=23,9%)和血栓性微血管病(=22,8%)。在非 MGRS 组中,膜性肾病是最常见的诊断(=171,40%)。在多变量逻辑回归模型中,异常血清游离轻链比、年龄较大和蛋白尿较多与 MGRS 独立相关。
在患有单克隆丙种球蛋白病的中国患者中,单克隆 Ig 淀粉样变性是 MGRS 的主要原因。异常游离轻链比、年龄较大和蛋白尿较多与 MGRS 相关。