Pathogenesis, histopathologic findings and treatment modalities of lipoprotein glomerulopathy: A review

Abstract Lipoprotein glomerulopathy (LPG) is an uncommon cause of nephrotic syndrome and/or kidney failure. At microscopy, LPG is characterized by the presence of lipoprotein thrombi in dilated glomerular capillaries due to different ApoE mutations. ApoE gene is located on chromosome 19q13.2, and can be identified in almost all serum lipoproteins. ApoE works as a protective factor in atherosclerosis due its interaction with receptor-mediated lipoprotein clearance and cholesterol receptor. Most common polymorphisms include ApoE2/2, ApoE3/2, ApoE3/3, ApoE4/2, ApoE4/3, and ApoE4/4. All age-groups can be affected by LPG, with a discrete male predominance. Compromised patients typically reveal dyslipidemia, type III hyperlipoproteinemia, and proteinuria. LPG treatment includes fenofibrate, antilipidemic drugs, steroids, LDL aphaeresis, plasma exchange, antiplatelet drugs, anticoagulants, urokinase, and renal transplantation. Recurrence in kidney graft suggests a pathogenic component(s) of extraglomerular humoral complex resulting from abnormal lipoprotein metabolism and presumably associated to ApoE.摘要 脂蛋白肾小球病（Lipoprotein glomerulopathy, LPG）是引发肾病综合征和/或肾衰竭的罕见病因。显微镜下，LPG以因不同载脂蛋白E（Apolipoprotein E, ApoE）突变导致的扩张肾小球毛细血管内出现脂蛋白血栓为特征。ApoE基因定位于19号染色体q13.2区域，几乎可在所有血清脂蛋白中被检出。ApoE可通过与受体介导的脂蛋白清除及胆固醇受体相互作用，在动脉粥样硬化中发挥保护作用。常见多态性包括ApoE2/2、ApoE3/2、ApoE3/3、ApoE4/2、ApoE4/3及ApoE4/4。所有年龄段人群均可罹患LPG，男性患者略占多数。受累患者通常表现为血脂异常、Ⅲ型高脂蛋白血症及蛋白尿。LPG的治疗手段包括非诺贝特、降脂药物、糖皮质激素、低密度脂蛋白（Low-Density Lipoprotein, LDL）吸附术、血浆置换、抗血小板药物、抗凝药物、尿激酶及肾移植。肾移植术后复发提示存在由异常脂蛋白代谢产生的肾小球外体液复合物致病成分，推测该成分与ApoE相关。