膜增生性肾小球肾炎

膜增生性肾小球肾炎
(Membranoproliferative glomerulonephritis )
膜增生性肾小球肾炎; (Membranoproliferative glomerulonephritis )
	显微照片指出肾小球中"膜增生性肾小球肾炎"之系膜基质增多，及系膜细胞结构增多。肾活检。PAS染色法。
类型	肾小球肾炎[]、proliferative glomerulonephritis[]、疾病
分类和外部资源
医学专科	泌尿外科
ICD-10	N00-N08 with .5 and .6 suffix
ICD-9-CM	581.2, 582.2, 583.2
OMIM	609814 305800
DiseasesDB	34457
MedlinePlus	000475
eMedicine	med/887
MeSH	D015432
GeneReviews	致密物沉积病/膜增生性肾炎II型.;
Orphanet	93571
	[编辑此条目的维基数据]

膜增生性肾小球肾炎（Membranoproliferative glomerulonephritis，MPGN），也被称为系膜毛细管性肾小球肾炎（mesangiocapillary glomerulonephritis），^[1] 是一种类型的肾小球肾炎引起的肾脏"肾小球系膜及基底膜(GBM)"增厚沉积，激活补体系统和破坏肾小球。

MPGN之肾炎综合征的"原发性肾起因"(primary renal cause)于儿童约占4％而成年人约7％。

它不应该与膜性肾小球肾炎相混淆，"膜性肾小球肾炎"是处于基底膜增厚的状态下，而其系膜是没有此种情况的。

病理

膜增生性肾小球肾炎涉及在肾小球内系膜区的沉积物。

它也是主要的C型肝炎相关性肾病。

该组织形态学的鉴别诊断(differential diagnosis)包括移植肾小球病(Transplant glomerulopathy)及血栓性微血管病(Thrombotic microangiopathy)。

外观

肾小球基底膜（GBM）是重建在沉积物之上，在显微镜下造成了电车轨道现象（英语：Tram track (medicine)）的出现。^[2] 致肾小球系膜细胞数增加。^[3]

类型

有三种类型的MPGN，但是这种分类是比较陈旧的做法、由于MPGN的发病原因模式已经变得较清楚。

I型

I型是迄今为止最常见的、是通过免疫复合物(Immune complex)的沉积在肾里所引起的。它的特点是内皮下和系膜免疫沉积物。

它被认为是与经典补体途径(Classical complement pathway)相关联。^[4]

II型

II型优选的名称是"致密物沉积病(dense deposit disease)。^[5] 大多数情况下致密物沉积病不会显示膜增生性图案，^[6] 一份2012年的论文认为DDD是与C3肾小球肾炎(Glomerulonephritis)是有所连续，^[7] 其中一个原因是I型到III的分类系统渐渐失去支持。

大多数的情况下，与另类补体途径(Alternative complement pathway)失调有关。^[8]^[9]

MPGN-II的自发缓解是罕见的，大约一半受MPGN-II影响的人在10年之内将发展到终末期肾脏疾病。^[10]

在许多情况下，MPGN-II的病情会发展为玻璃膜疣(drusen/脉络膜基底层透明小疣)，而玻璃膜疣是由位于眼睛视网膜色素上皮细胞( retinal pigment epithelium/RPE)下方的布鲁赫膜(Bruch's membrane)之相同沉积物所引起的。随着时间的推移视力也会变差且有"视网膜下新生血管膜"(subretinal neovascular membranes)、黄斑区脱离(macular detachment)，以及中心性浆液性脉络膜视网膜病变的发展。^[11]

(参见：依库珠单抗#致密物沉积病 (DDD)(Eculizumab))

III型

III型是非常罕见的，它的特征在于皮下沉积物的混合及I型疾病的典型病理结果。

候选基因已被确定在1号染色体。^[12]

补体成分3(Complement component 3)是根据免疫荧光(Immunofluorescence)来观察。^[13]

注释

^ Colville D, Guymer R, Sinclair RA, Savige J. Visual impairment caused by retinal abnormalities in mesangiocapillary (membranoproliferative) glomerulonephritis type II ("dense deposit disease"). Am. J. Kidney Dis. August 2003, 42 (2): E2–5 [2014-07-30]. PMID 12900843. doi:10.1016/S0272-6386(03)00665-6. （原始内容存档于2020-08-10）.
^ Membranoproliferative_glomerulonephritis_type_I of the Kidney. [2008-11-25]. （原始内容存档于2006-09-10）.
^ Renal Pathology. [2008-11-25]. （原始内容存档于2017-09-01）.
^ West CD, McAdams AJ. Glomerular paramesangial deposits: association with hypocomplementemia in membranoproliferative glomerulonephritis types I and III. Am. J. Kidney Dis. March 1998, 31 (3): 427–34 [2014-11-03]. PMID 9506679. doi:10.1053/ajkd.1998.v31.pm9506679. （原始内容存档于2020-08-10）.
^ Final Diagnosis — Case 148. [2008-11-25]. （原始内容存档于2020-11-29）.
^ Patrick D Walker, Franco Ferrario, Kensuke Joh, Stephen M Bonsib. Dense deposit disease is not a membranoproliferative glomerulonephritis. Modern Pathology. 2007, 20 (6): 605–616. PMID 17396142. doi:10.1038/modpathol.3800773.
^ Sethi, Fervenza, S, FC. Membranoproliferative glomerulonephritis - a new look at an old entity. N Engl J Med. 2012, 366 (12): 1119–1131 [2014-11-03]. PMID 22435371. doi:10.1056/NEJMra1108178. （原始内容存档于2019-10-31）.
^ Rose KL, Paixao-Cavalcante D, Fish J; et al. Factor I is required for the development of membranoproliferative glomerulonephritis in factor H-deficient mice. J. Clin. Invest. February 2008, 118 (2): 608–18. PMC 2200299 . PMID 18202746. doi:10.1172/JCI32525.
^ Licht C, Schlötzer-Schrehardt U, Kirschfink M, Zipfel PF, Hoppe B. MPGN II--genetically determined by defective complement regulation?. Pediatr. Nephrol. January 2007, 22 (1): 2–9. PMID 17024390. doi:10.1007/s00467-006-0299-8.
^ Swainson CP, Robson JS, Thomson D, MacDonald MK. Mesangiocapillary glomerulonephritis: a long-term study of 40 cases. J. Pathol. 1983, 141 (4): 449–68. PMID 6363655. doi:10.1002/path.1711410404.
^ Colville D, Guymer R, Sinclair RA, Savige J. Visual impairment caused by retinal abnormalities in mesangiocapillary (membranoproliferative) glomerulonephritis type II ("dense deposit disease"). Am. J. Kidney Dis. 2003, 42 (2): E2–5 [2014-07-30]. PMID 12900843. doi:10.1016/S0272-6386(03)00665-6. （原始内容存档于2020-08-10）.
^ Neary JJ, Conlon PJ, Croke D; et al. Linkage of a gene causing familial membranoproliferative glomerulonephritis type III to chromosome 1. J. Am. Soc. Nephrol. August 2002, 13 (8): 2052–7 [2014-11-03]. PMID 12138136. doi:10.1097/01.ASN.0000022006.49966.F8. （原始内容存档于2020-05-30）.
^ Neary J, Dorman A, Campbell E, Keogan M, Conlon P. Familial membranoproliferative glomerulonephritis type III. Am. J. Kidney Dis. July 2002, 40 (1): E1 [2014-11-03]. PMID 12087587. doi:10.1053/ajkd.2002.33932. （原始内容存档于2020-08-10）.

参见

病毒性出血热

外部链接

Glomerulonephritis, Membranoproliferative Types I,II,III 于 eMedicine
Corchado, Johnny Cruz; Smith, Richard JH. Dense Deposit Disease/Membranoproliferative Glomerulonephritis Type II. Pagon RA, Bird TD, Dolan CR; et al (编). GeneReviews. Seattle WA: University of Washington. July 2007 [2014-07-30]. PMID 20301598. （原始内容存档于2020-12-01）.
Membranoproliferative_GN （页面存档备份，存于互联网档案馆） at Nephropathology tutorial （页面存档备份，存于互联网档案馆）
MP GN Pathophysiology （页面存档备份，存于互联网档案馆） discusses the nephritic auto-antibodies/factors

[pmid12900843-1] Colville D, Guymer R, Sinclair RA, Savige J. Visual impairment caused by retinal abnormalities in mesangiocapillary (membranoproliferative) glomerulonephritis type II ("dense deposit disease"). Am. J. Kidney Dis. August 2003, 42 (2): E2–5 [2014-07-30]. PMID 12900843. doi:10.1016/S0272-6386(03)00665-6. （原始内容存档于2020-08-10）.

[2] Membranoproliferative_glomerulonephritis_type_I of the Kidney. [2008-11-25]. （原始内容存档于2006-09-10）.

[3] Renal Pathology. [2008-11-25]. （原始内容存档于2017-09-01）.

[pmid9506679-4] West CD, McAdams AJ. Glomerular paramesangial deposits: association with hypocomplementemia in membranoproliferative glomerulonephritis types I and III. Am. J. Kidney Dis. March 1998, 31 (3): 427–34 [2014-11-03]. PMID 9506679. doi:10.1053/ajkd.1998.v31.pm9506679. （原始内容存档于2020-08-10）.

[urlFinal_Diagnosis_--_Case_148-5] Final Diagnosis — Case 148. [2008-11-25]. （原始内容存档于2020-11-29）.

[6] Patrick D Walker, Franco Ferrario, Kensuke Joh, Stephen M Bonsib. Dense deposit disease is not a membranoproliferative glomerulonephritis. Modern Pathology. 2007, 20 (6): 605–616. PMID 17396142. doi:10.1038/modpathol.3800773.

[Sethi2012-7] Sethi, Fervenza, S, FC. Membranoproliferative glomerulonephritis - a new look at an old entity. N Engl J Med. 2012, 366 (12): 1119–1131 [2014-11-03]. PMID 22435371. doi:10.1056/NEJMra1108178. （原始内容存档于2019-10-31）.

[pmid18202746-8] Rose KL, Paixao-Cavalcante D, Fish J; et al. Factor I is required for the development of membranoproliferative glomerulonephritis in factor H-deficient mice. J. Clin. Invest. February 2008, 118 (2): 608–18. PMC 2200299 . PMID 18202746. doi:10.1172/JCI32525.

[pmid17024390-9] Licht C, Schlötzer-Schrehardt U, Kirschfink M, Zipfel PF, Hoppe B. MPGN II--genetically determined by defective complement regulation?. Pediatr. Nephrol. January 2007, 22 (1): 2–9. PMID 17024390. doi:10.1007/s00467-006-0299-8.

[10] Swainson CP, Robson JS, Thomson D, MacDonald MK. Mesangiocapillary glomerulonephritis: a long-term study of 40 cases. J. Pathol. 1983, 141 (4): 449–68. PMID 6363655. doi:10.1002/path.1711410404.

[11] Colville D, Guymer R, Sinclair RA, Savige J. Visual impairment caused by retinal abnormalities in mesangiocapillary (membranoproliferative) glomerulonephritis type II ("dense deposit disease"). Am. J. Kidney Dis. 2003, 42 (2): E2–5 [2014-07-30]. PMID 12900843. doi:10.1016/S0272-6386(03)00665-6. （原始内容存档于2020-08-10）.

[pmid12138136-12] Neary JJ, Conlon PJ, Croke D; et al. Linkage of a gene causing familial membranoproliferative glomerulonephritis type III to chromosome 1. J. Am. Soc. Nephrol. August 2002, 13 (8): 2052–7 [2014-11-03]. PMID 12138136. doi:10.1097/01.ASN.0000022006.49966.F8. （原始内容存档于2020-05-30）.

[13] Neary J, Dorman A, Campbell E, Keogan M, Conlon P. Familial membranoproliferative glomerulonephritis type III. Am. J. Kidney Dis. July 2002, 40 (1): E1 [2014-11-03]. PMID 12087587. doi:10.1053/ajkd.2002.33932. （原始内容存档于2020-08-10）.

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