Dysproteinemia and the Kidney: Core Curriculum 2019
Dysproteinemia and the Kidney: Core Curriculum 2019
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DOI：
10.1053/j.ajkd.2019.04.029
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发表时间：
2019-12-01
影响因子：
13.2
通讯作者：
Leung, Nelson
中科院分区：
文献类型：
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作者：
Hogan, Jonathan J.;Alexander, Mariam Priya;Leung, Nelson
当 B 细胞或浆细胞克隆产生导致肾脏损伤的致病性单克隆免疫球蛋白或轻链时，就会发生蛋白异常性肾病。这些疾病的临床表现包括肾病范围内的蛋白尿或肾功能保留的镜下血尿、严重肾病综合征、严重急性肾损伤或急进性肾小球肾炎。这些单克隆免疫球蛋白可引起多种组织学损伤模式，包括管型肾病、肾小球和肾小管沉积病、淀粉样变性和炎性肾小球肾炎。潜在的克隆性疾病可能符合明显的多发性骨髓瘤或系统性淋巴瘤的标准。近年来，人们对在较小克隆血浆和 B 细胞群体中发生的蛋白异常肾病的认识和研究有所增加，这些疾病被归类为具有肾脏意义的单克隆丙种球蛋白病。无论克隆细胞负荷如何，治疗的目标是通过根除潜在的克隆来实现血液学反应（即单克隆蛋白的改善或解决）。器官特异性反应取决于血液学反应的实现。如果没有适当的治疗，许多这些疾病都与进行性肾病和终末期肾病的高发病率有关。在本期 AJKD 肾病学核心课程中，我们回顾了异常蛋白血症肾病的发病机制、诊断和治疗。
Dysproteinemic kidney diseases occur when B- or plasma cell clones produce pathogenic monoclonal immunoglobulins or light chains that cause kidney damage. The clinical presentation of these disorders ranges from sub-nephrotic-range proteinuria or microscopic hematuria with preserved kidney function to severe nephrotic syndrome to severe acute kidney injury or rapidly progressive glomerulonephritis. These monoclonal immunoglobulins can cause a variety of histologic patterns of injury, including cast nephropathy, glomerular and tubular deposition diseases, amyloidosis, and inflammatory glomerulonephritis. The underlying clonal disorder may meet criteria for overt multiple myeloma or systemic lymphoma. In recent years, there has been increased recognition and study of dysproteinemic kidney diseases that occur in the setting of smaller clonal plasma and B-cell populations, which are classified as monoclonal gammopathies of renal significance. Regardless of clonal cell burden, the goal of treatment is to achieve a hematologic response (ie, improvement or resolution of the monoclonal protein) by eradicating the underlying clone. Organ-specific responses are dependent on achieving hematologic response. Without appropriate treatment, many of these disorders are associated with high rates of progressive kidney disease and end-stage kidney disease. In this installment of AJKD's Core Curriculum in Nephrology, we review the pathogenesis, diagnosis, and treatment of dysproteinemic kidney diseases.