Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China.
Institute of Nephrology, Peking University, Beijing, China.
Nephrology (Carlton). 2020 Mar;25(3):202-211. doi: 10.1111/nep.13633. Epub 2019 Aug 1.
To analysis the pathological spectrum and prognosis of monoclonal gammopathy of renal significance (MGRS) patients.
Patients with renal biopsy-proven MGRS from 1999 to 2017 in Peking University First Hospital were included, clinical data, renal pathology type, treatment and prognosis were collected.
One hundred and eighty-seven patients were enrolled, accounting for 0.7% of renal biopsies. Seventy-seven per cent of the MGRS patients were amyloidosis. Eighteen patients (9.6%) were monoclonal immunoglobulin deposition disease. Others included 10 patients (5.3%) with proliferative glomerulonephritis with monoclonal immunoglobulin (G) deposits, seven patients (3.7%) with cryoglobulinaemic glomerulonephritis, five patients (2.6%) with light chain proximal tubulopathy, two patients (1.1%) with fibrillary disease and one patient (0.5%) with C3 glomerulonephritis. Sixty-three per cent were treated with chemotherapy and/or stem cell transplantation. The mean follow-up time was 27 ± 32 months. One patient developed multiple myeloma at 17-month during follow-up. At the end of follow-up, 61 patients (33%) died, and 47 patients (25%) reached end-stage renal disease (ESRD). For the 144 amyloid patients, low estimated glomerular filtration rate (eGFR), decreased blood pressure, presence of cardiac involvement and absence of chemotherapy or high-dose melphalan/autologous peripheral blood stem cell transplantation were identified as independent risk factors for death. Low eGFR, decreased blood pressure, and presence of cardiac involvement were identified as independent risk factors for ESRD. For the 43 non-amyloid patients, no factor was identified for the risk of death. Low eGFR was identified as independent risk factor for ESRD.
MGRS was an uncommon form of hematologic disorder related renal injury with a wide spectrum of pathologic lesions, and amyloidosis was the most common type. Treatment with chemotherapy and/or high-dose melphalan/autologous peripheral blood stem cell transplantation improved amyloid patients' survival.
分析单克隆免疫球蛋白相关性肾损害（MGRS）患者的病理谱和预后。
纳入 1999 年至 2017 年在北京大学第一医院经肾活检证实为 MGRS 的患者，收集临床资料、肾脏病理类型、治疗和预后。
共纳入 187 例患者，占肾活检的 0.7%。77%的 MGRS 患者为淀粉样变性。18 例（9.6%）为单克隆免疫球蛋白沉积病。其他包括 10 例（5.3%）伴单克隆免疫球蛋白（G）沉积的增生性肾小球肾炎、7 例（3.7%）冷球蛋白血症性肾小球肾炎、5 例（2.6%）轻链近端肾小管病、2 例（1.1%）纤维样疾病和 1 例（0.5%）C3 肾小球肾炎。63%的患者接受化疗和/或干细胞移植治疗。平均随访时间为 27±32 个月。1 例患者在随访 17 个月时发展为多发性骨髓瘤。随访结束时，61 例（33%）患者死亡，47 例（25%）患者达到终末期肾病（ESRD）。对于 144 例淀粉样变性患者，低估计肾小球滤过率（eGFR）、血压降低、心脏受累和无化疗或大剂量马法兰/自体外周血干细胞移植被确定为死亡的独立危险因素。低 eGFR、血压降低和心脏受累被确定为 ESRD 的独立危险因素。对于 43 例非淀粉样变性患者，未发现死亡的危险因素。低 eGFR 是 ESRD 的独立危险因素。
MGRS 是一种罕见的与血液系统疾病相关的肾损伤形式，病理病变谱广泛，淀粉样变性最常见。化疗和/或大剂量马法兰/自体外周血干细胞移植治疗可改善淀粉样变性患者的生存。
