Barrett Caroline M F, Troxell Megan L, Larsen Christopher P, Houghton Donald C
Department of Pathology, Oregon Health and Science University, L471, 3181 SW Sam Jackson Park Rd, Portland, OR, 97239, USA.
Int Urol Nephrol. 2014 May;46(5):963-71. doi: 10.1007/s11255-013-0593-x. Epub 2013 Nov 12.
The coexistence of membranous glomerulonephritis (MGN) and necrotizing and crescentic glomerulonephritis (NCGN) is an unusual finding in a renal biopsy except in lupus nephritis. Little is known about whether these lesions are causally related in any clinical setting.
We reviewed the pathology, presentation, and clinical course of 13 non-lupus patients with combined MGN and NCGN in native kidney biopsies (nine females, four males; median age 69 years), with particular attention to evidence of secondary MGN. Additional IgG subclass and phospholipase A2 receptor (PLA2R) immunofluorescence studies were conducted in seven cases.
Eight biopsies were pauci-immune other than the capillary wall deposits of MGN; one patient had a non-lupus immune complex disease, and four had mesangial deposits, including one with rare subendothelial deposits. None had anti-glomerular basement membrane disease. IgG4 was dominant or codominant in the capillary wall deposits in three cases and virtually absent in four; PLA2R was positive in two cases, and negative in five. Seven patients were judged to have secondary MGN, including five of eight ANCA+ patients. Twelve patients were treated with combinations of steroids, cyclophosphamide, rituximab, followed by durable response in seven and relentless progression to end stage renal disease in four.
Secondary MGN occurs with higher frequency in ANCA-positive NCGN than in the general MGN population. A causal relationship between MGN and NCGN was not established in any patient, but circumstances suggest a common cause in several, including immune complex disease, drug reaction and paraneoplastic syndrome.
除狼疮性肾炎外，膜性肾小球肾炎（MGN）与坏死性新月体性肾小球肾炎（NCGN）并存是肾活检中不常见的发现。在任何临床情况下，对于这些病变是否存在因果关系知之甚少。
我们回顾了13例非狼疮患者的病理、临床表现及临床病程，这些患者在肾活检中同时存在MGN和NCGN（9例女性，4例男性；中位年龄69岁），特别关注继发性MGN的证据。对7例患者进行了额外的IgG亚类和磷脂酶A2受体（PLA2R）免疫荧光研究。
除MGN的毛细血管壁沉积物外，8例活检显示寡免疫；1例患者患有非狼疮性免疫复合物疾病，4例有系膜沉积物，其中1例有罕见的内皮下沉积物。均无抗肾小球基底膜病。3例患者的毛细血管壁沉积物中IgG4占主导或共显性，4例几乎不存在；2例PLA2R阳性，5例阴性。7例患者被判定为继发性MGN，包括8例ANCA阳性患者中的5例。12例患者接受了类固醇、环磷酰胺、利妥昔单抗联合治疗，7例病情持久缓解，4例病情持续进展至终末期肾病。
继发性MGN在ANCA阳性的NCGN中出现的频率高于一般MGN人群。在任何患者中均未确立MGN与NCGN之间的因果关系，但在一些病例中情况提示存在共同病因，包括免疫复合物疾病、药物反应和副肿瘤综合征。
