Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Semin Nephrol. 2011 Jul;31(4):341-8. doi: 10.1016/j.semnephrol.2011.06.005.
Membranoproliferative glomerulonephritis (MPGN) is a pattern of injury that results from subendothelial and mesangial deposition of Igs caused by persistent antigenemia and/or circulating immune complexes. The common causes of Ig-mediated MPGN include chronic infections, autoimmune diseases, and monoclonal gammopathy/dysproteinemias. On the other hand, MPGN also can result from subendothelial and mesangial deposition of complement owing to dysregulation of the alternative pathway (AP) of complement. Complement-mediated MPGN includes dense deposit disease and proliferative glomerulonephritis with C3 deposits. Dysregulation of the AP of complement can result from genetic mutations or development of autoantibodies to complement regulating proteins with ensuing dense deposit disease or glomerulonephritis with C3 deposits. We propose a new histologic classification of MPGN and classify MPGN into 2 major groups: Ig-mediated and complement-mediated. MPGN that is Ig-mediated should lead to work-up for infections, autoimmune diseases, and monoclonal gammopathy. On the other hand, complement-mediated MPGN should lead to work-up of the AP of complement. Initial AP screening tests should include serum membrane attack complex levels, an AP functional assay, and a hemolytic assay, followed by tests for mutations and autoantibodies to complement-regulating proteins.
膜增生性肾小球肾炎 (MPGN) 是一种损伤模式，由持续性抗原血症和/或循环免疫复合物引起的内皮下和系膜沉积 Ig 导致。Ig 介导的 MPGN 的常见原因包括慢性感染、自身免疫性疾病和单克隆丙种球蛋白病/蛋白血症。另一方面，MPGN 也可能由于补体替代途径 (AP) 的失调导致补体在内皮下和系膜沉积。补体介导的 MPGN 包括致密物沉积病和 C3 沉积的增殖性肾小球肾炎。补体 AP 的失调可由基因突变或针对补体调节蛋白的自身抗体的发展引起，随后发生致密物沉积病或 C3 沉积的肾小球肾炎。我们提出了一种新的 MPGN 组织学分类，并将 MPGN 分为 2 大主要组：Ig 介导和补体介导。由 Ig 介导的 MPGN 应进行感染、自身免疫性疾病和单克隆丙种球蛋白病的检查。另一方面，补体介导的 MPGN 应进行补体 AP 的检查。初始 AP 筛选测试应包括血清膜攻击复合物水平、AP 功能测定和溶血测定，然后是补体调节蛋白的突变和自身抗体测试。
