Nargund Preeti, Kambham Neeraja, Mehta Kshama, Lafayette Richard A
Clin Nephrol. 2015 Dec;84(6):323-30. doi: 10.5414/CN108619.
A recent classification of membranoproliferative glomerulonephritis (MPGN) utilizes the presence of immunoglobulin and complements to simplify diagnosis and point towards disease etiology. Here, we evaluate a historic cohort of patients with idiopathic MPGN using the new classification system and correlate it with clinical outcome.
We identified 281 patients diagnosed with MPGN at Stanford from 2000 to 2012. Patients with hepatitis, systemic lupus erythematosis, lymphomas, and plasma cell dyscrasias were excluded. The clinicopathologic findings of the remaining 71 patients were further analyzed and differences between immunoglobulin dominant (IM) and complement dominant (CM) disease were evaluated.
Using the new classification system, 51 subjects were characterized as CM MPGN and 20 as IM MPGN. In the CM MPGN group, there was a non-significant trend towards lower proteinuria but higher serum creatinine values. At biopsy, most subjects had less than 50% global sclerosis or cortical scarring. The majority of subjects in the CM MPGN group (41%) had C3 nephropathy while 60% of subjects in IM MPGN group had C3 dominant disease. Treatment and outcomes: During follow-up (median 2 years), 20 patients reached a clinical end point of dialysis or death. The mean creatinine was significantly higher while the baseline proteinuria also trended slightly higher. Prednisone use was statistically higher in the survivor group.
Our study highlights the clinicopathological features of patients with biopsy proven MPGN with no known etiological factors and sheds some light on the incidence and outcomes of various categories of MPGN under the new criteria, including MPGN with "dominant C3" deposits, rapidly becoming a descriptive diagnosis.
最近对膜增生性肾小球肾炎(MPGN)的分类利用免疫球蛋白和补体的存在来简化诊断并指向疾病病因。在此,我们使用新的分类系统评估一组特发性MPGN患者的历史队列,并将其与临床结果相关联。
我们确定了2000年至2012年在斯坦福大学被诊断为MPGN的281例患者。排除患有肝炎、系统性红斑狼疮、淋巴瘤和浆细胞异常的患者。对其余71例患者的临床病理结果进行进一步分析,并评估免疫球蛋白为主(IM)和补体为主(CM)疾病之间的差异。
使用新的分类系统,51名受试者被归类为CM MPGN,20名被归类为IM MPGN。在CM MPGN组中,蛋白尿较低但血清肌酐值较高有不显著的趋势。活检时,大多数受试者的全球硬化或皮质瘢痕小于50%。CM MPGN组中的大多数受试者(41%)患有C3肾病,而IM MPGN组中的60%受试者患有以C3为主的疾病。治疗与结果:在随访期间(中位时间2年),20例患者达到了透析或死亡的临床终点。平均肌酐显著更高,而基线蛋白尿也略有更高的趋势。幸存者组中泼尼松的使用在统计学上更高。
我们的研究突出了经活检证实的无已知病因的MPGN患者的临床病理特征,并根据新标准对各类MPGN的发病率和结果提供了一些见解,包括具有“主要C3”沉积物的MPGN,其正迅速成为一种描述性诊断。