蛋白异常血症相关肾病:临床病理相关性

M. Mm, H. Sousa, M. Góis, F. Nolasco, Costa Ll., E. Soares
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摘要

引言:与蛋白质异常血症相关的肾脏疾病可能有不同的临床和组织学表现,但并非所有单克隆抗体血症患者都患有肾意义单克隆抗体血症(MGRS),有些患者还有其他引起肾脏病变的原因。因此,肾活检对做出这种诊断至关重要。我们进行了一项回顾性研究,目的是:1。识别与蛋白代谢异常相关的肾脏病变;2.建立具有这些病变的患者的临床病理相关性。确定肾脏和患者生存预测因素。方法:对2015年1月至2020年2月在葡萄牙三家医院进行的肾活检进行回顾性、观察性图表审查,共有39名患者患有与单克隆gammopathy相关的肾脏病变。结果:发现的三种主要的异常蛋白血症肾病是铸造肾病、AL淀粉样变性和单克隆免疫球蛋白沉积病(MIDD),它们之间有不同的特征。只有三名患者符合肾意义单克隆抗体肾病(MGRS)的标准。在治疗方面,我们证实我们的大多数患者都接受了化疗。不幸的是,只有4人部分或完全康复。自肾活检以来的平均肾脏生存期为29,23个月,自诊断以来的平均患者生存期为24,46个月。一些与较低生存率相关的临床和病理特征:急性肾小管坏死、铸造肾病、血栓性微血管病(TMA)、血红蛋白和估计肾小球滤过率(eGFR)。既往肾脏病随访与较高的肾脏存活率相关。只有eGFR与较低的患者生存率相关。
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Dysproteinemia-Associated Kidney Diseases: Clinicopathological Correlations
Introduction: Dysproteinemia-associated kidney diseases can have diverse clinical and histological presentation but not all patients with monoclonal gammopathy have Monoclonal Gammopathy of Renal Significance (MGRS) and some have other causes for kidney lesions. Therefore, kidney biopsy is essential to make this diagnosis. We made a retrospective study, which aimed to: 1. Identify dysproteinemiaassociated kidney lesions; 2. Establish clinicopathological correlations of patients with those lesions and 3. Identify kidney and patient survival predictors. Methods: A retrospective, observational chart review of kidney biopsies performed, between January 2015 and February 2020, in three Portuguese Hospitals, to a total of 39 patients, with kidney lesions associated with monoclonal gammopathy, was undertaken. Results: The three main dysproteinemic kidney diseases identified were cast nephropathy, AL amyloidosis and Monoclonal Immunoglobulin Deposition Disease (MIDD), with different features among them. Only three patients fulfilled the criteria to Monoclonal Gammopathy of Renal Significance (MGRS). In regard to treatment, we verified that most of our patients were treated with chemotherapy. Unfortunately, only four recovered, either partially or completely. The mean kidney survival since kidney biopsy was 29,23 months and the mean patient survival since diagnosis was 24,46 months. Some clinical and pathologic features correlated to lowerkidney survival: acute tubular necrosis, cast nephropathy, Thrombotic Microangiopathy (TMA), haemoglobin and estimated Glomerular Filtration Rate (eGFR). Previous Nephrology follow-up correlated with higher kidney survival. Only eGFR was associated with lowerpatient survival.
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