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.

当B细胞或浆细胞克隆产生致病性单克隆免疫球蛋白或轻链导致肾脏损害时，就会发生蛋白质失调性肾脏疾病。这些疾病的临床表现包括亚肾病范围蛋白尿或保留肾功能的镜下血尿，严重肾病综合征，严重急性肾损伤或快速进展性肾小球肾炎。这些单克隆免疫球蛋白可导致多种组织学损伤模式，包括铸型肾病，肾小球和肾小管沉积疾病，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。最近几年，在较小的克隆血浆和B细胞群的情况下发生的脂蛋白血症性肾脏疾病的认识和研究得到了提高，这些疾病被归类为具有肾脏意义的单克隆血友病。不管克隆细胞的负担如何，治疗的目标都是通过消除潜在的克隆来实现血液学应答（即单克隆蛋白的改善或分离）。器官特异性反应取决于实现血液学反应。如果没有适当的治疗，这些疾病中有许多与进展性肾脏疾病和终末期肾脏疾病的发生率高有关。在本期的 通过消除基础克隆来改善或分离单克隆蛋白）。器官特异性反应取决于实现血液学反应。如果没有适当的治疗，这些疾病中有许多与进展性肾脏疾病和终末期肾脏疾病的发生率高有关。在本期的 通过消除基础克隆来改善或分离单克隆蛋白）。器官特异性反应取决于实现血液学反应。如果没有适当的治疗，这些疾病中有许多与进展性肾脏疾病和终末期肾脏疾病的发生率高有关。在本期的AJKD的肾脏病学核心课程，我们回顾了蛋白血症性肾脏疾病的发病机理，诊断和治疗。