Rudnicki Michael
Department of Internal Medicine IV-Nephrology and Hypertension, Medical University Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria.
Biomed Res Int. 2017;2017:2180508. doi: 10.1155/2017/2180508. Epub 2017 May 9.
Membranoproliferative glomerulonephritis (MPGN) is a histological pattern of injury resulting from predominantly subendothelial and mesangial deposition of immunoglobulins or complement factors with subsequent inflammation and proliferation particularly of the glomerular basement membrane. Recent classification of MPGN is based on pathogenesis dividing MPGN into immunoglobulin-associated MPGN and complement-mediated C3 glomerulonephritis (C3GN) and dense deposit disease (DDD). Current guidelines suggest treatment with steroids, cytotoxic agents with or without plasmapheresis only for subjects with progressive disease, that is, nephrotic range proteinuria and decline of renal function. Rituximab, a chimeric B-cell depleting anti-CD20 antibody, has emerged in the last decade as a treatment option for patients with primary glomerular diseases such as minimal change disease, focal-segmental glomerulosclerosis, or idiopathic membranous nephropathy. However, data on the use of rituximab in MPGN, C3GN, and DDD are limited to case reports and retrospective case series. Patients with immunoglobulin-associated and idiopathic MPGN who were treated with rituximab showed partial and complete responses in the majorities of cases. However, rituximab was not effective in few cases of C3GN and DDD. Despite promising results in immunoglobulin-associated and idiopathic MPGN, current evidence on this treatment remains weak, and controlled and prospective data are urgently needed.
膜增生性肾小球肾炎(MPGN)是一种组织学损伤模式,主要由免疫球蛋白或补体因子在内皮下和系膜沉积,随后引发炎症和增殖,尤其是肾小球基底膜的增殖。MPGN的最新分类基于发病机制,将MPGN分为免疫球蛋白相关的MPGN和补体介导的C3肾小球肾炎(C3GN)及致密物沉积病(DDD)。目前的指南建议,仅对病情进展的患者,即出现肾病范围蛋白尿和肾功能下降的患者,使用类固醇、细胞毒性药物,可联合或不联合血浆置换进行治疗。利妥昔单抗是一种嵌合型耗竭B细胞的抗CD20抗体,在过去十年中已成为治疗原发性肾小球疾病(如微小病变病、局灶节段性肾小球硬化或特发性膜性肾病)患者的一种治疗选择。然而,关于利妥昔单抗在MPGN、C3GN和DDD中的应用数据仅限于病例报告和回顾性病例系列。接受利妥昔单抗治疗的免疫球蛋白相关和特发性MPGN患者在大多数病例中显示出部分和完全缓解。然而,利妥昔单抗在少数C3GN和DDD病例中无效。尽管在免疫球蛋白相关和特发性MPGN中取得了有前景的结果,但目前关于这种治疗方法的证据仍然不足,迫切需要对照和前瞻性数据。