Membranoproliferative glomerulonephritis and C3 glomerulopathy: resolving the confusion.
Membranoproliferative glomerulonephritis and C3 glomerulopathy: resolving the confusion.
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DOI：
10.1038/ki.2011.399
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发表时间：
2012-03
影响因子：
19.6
通讯作者：
中科院分区：
文献类型：
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膜增生性肾小球肾炎（MPGN）表示肾小球损伤的一般模式，很容易通过光学显微镜识别。通过进一步的研究，MPGN亚组是可能的。例如，电子显微镜可分辨电子致密沉积的差异，这些差异通常被称为MPGN I型（MPGN I）、MPGN II和MPGN III，而免疫荧光通常检测MPGN I和MPGN III中的免疫球蛋白，但不检测MPGN II。所有三种MPGN类型对补体成分3（C3）染色呈阳性。亚群导致了不必要的混乱，主要是因为免疫球蛋白阴性的MPGN I和MPGN III比以前更常见。与MPGN II（现在称为致密存款病）一起，免疫球蛋白阴性、C3阳性肾小球疾病属于C3肾小球病的范畴。免疫球蛋白阳性MPGN的评价应侧重于确定驱动慢性抗原血症或循环免疫复合物的潜在触发因素，以便开始疾病特异性治疗。相反，C3肾小球病的评价应侧重于补体级联，因为旁路途径和终末补体级联的失调是发病机制的基础。虽然目前没有针对C3肾小球病的疾病特异性治疗方法，但更好地了解其发病机制将为可能使用抗补体药物奠定基础。
Membranoproliferative glomerulonephritis (MPGN) denotes a general pattern of glomerular injury that is easily recognized by light microscopy. With additional studies, MPGN subgrouping is possible. For example, electron microscopy resolves differences in electron-dense deposition that are classically referred to as MPGN type I (MPGN I), MPGN II and MPGN III, while immunofluorescence typically detects immunoglobulins in MPGN I and MPGN III but not MPGN II. All three MPGN types stain positive for complement component 3 (C3). Subgrouping has led to unnecessary confusion primarily because immunoglobulin-negative MPGN I and MPGN III are more common than once recognized. Together with MPGN II, which is now called Dense Deposit Disease, immunoglobulin-negative, C3-positive glomerular diseases fall under the umbrella of C3 Glomerulopathies. The evaluation of immunoglobulin-positive MPGN should focus on identifying the underlying trigger driving the chronic antigenemia or circulating immune complexes in order to begin disease-specific treatment. The evaluation of C3 Glomerulopathies, in contrast, should focus on the complement cascade, as dysregulation of the alternative pathway and terminal complement cascade underlies pathogenesis. Although there are no disease-specific treatments currently available for C3 Glomerulopathies, a better understanding of their pathogenesis would set the stage for the possible use of anti-complement drugs.
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影响因子：
4
作者：
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通讯作者：
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DOI：
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发表时间：
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作者：
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DOI：
10.2215/cjn.07110810
发表时间：
2011-05-01
影响因子：
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作者：
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通讯作者：
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