Abstract：
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.
摘要：
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。在这次审查中,我们关注副蛋白介导的肾小球疾病。肾活检在诊断这些疾病中起着至关重要的作用，能够识别特定的组织学模式。这些病变分为有组织的(如淀粉样变性，免疫触病样肾小球病，原纤维性肾小球肾炎，冷球蛋白血症性肾小球肾炎，和单克隆结晶肾小球病）和非组织化沉积物（例如单克隆Ig沉积疾病和具有单克隆Ig沉积物的增生性肾小球肾炎），基于免疫荧光发现的特征和电子显微镜上沉积物的超微结构外观。这篇评论旨在提供一个更新，突出显示,并讨论临床病理方面，如定义，流行病学,临床表现，肾损伤的机制，组织学特征，和诊断程序。
