Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN.
Department of Medicine, Section of Nephrology, University of Chicago, Chicago, IL.
Adv Kidney Dis Health. 2024 Jul;31(4):358-373. doi: 10.1053/j.akdh.2024.02.005.
Paraproteinemias are a group of complex diseases associated with an overproduction of a monoclonal immunoglobulin that can cause a diversity of kidney disorders and end-organ damage. In this review, we focus on paraprotein-mediated glomerular diseases. Kidney biopsy plays a crucial role in diagnosing these disorders, enabling the identification of specific histological patterns. These lesions are categorized into organized (such as amyloidosis, immunotactoid glomerulopathy, fibrillary glomerulonephritis, cryoglobulinemic glomerulonephritis, and monoclonal crystalline glomerulopathies) and nonorganized deposits (such as monoclonal Ig deposition disease and proliferative glomerulonephritis with monoclonal Ig deposits) based on the characteristics of immunofluorescence findings and the ultrastructural appearance of deposits on electron microscopy. This review aims to provide an update, highlight, and discuss clinicopathological aspects such as definition, epidemiology, clinical manifestations, mechanisms of kidney injury, histological features, and diagnostic procedures.
副蛋白血症是一组与单克隆免疫球蛋白过度产生相关的复杂疾病，可导致多种肾脏疾病和终末器官损伤。在本综述中，我们重点关注副蛋白介导的肾小球疾病。肾脏活检在诊断这些疾病中起着至关重要的作用，能够识别出特定的组织学模式。这些病变根据免疫荧光发现的特征和电子显微镜下沉积物的超微结构表现，分为有组织（如淀粉样变性、免疫触须样肾小球病、纤维状肾小球肾炎、冷球蛋白血症性肾小球肾炎和单克隆晶体肾小球病）和无组织沉积物（如单克隆免疫球蛋白沉积病和伴有单克隆免疫球蛋白沉积的增殖性肾小球肾炎）。本综述旨在提供最新信息，强调并讨论定义、流行病学、临床表现、肾脏损伤机制、组织学特征和诊断程序等临床病理方面。
