fbpx
Wikipedia

Membranoproliferative glomerulonephritis

March 04, 2023

Membranoproliferative glomerulonephritis (MPGN) is a type of glomerulonephritis caused by deposits in the kidney glomerular mesangium and basement membrane (GBM) thickening,[2] activating complement and damaging the glomeruli.

Membranoproliferative glomerulonephritis
Other namesMesangiocapillary glomerulonephritis[1]
Micrograph of glomerulus in membranoproliferative glomerulonephritis with increased mesangial matrix and increased mesangial cellularity. Kidney biopsy. PAS stain.
SpecialtyNephrology

MPGN accounts for approximately 4% of primary renal causes of nephrotic syndrome in children and 7% in adults.[3]

It should not be confused with membranous glomerulonephritis, a condition in which the basement membrane is thickened, but the mesangium is not.

Type

There are three types of MPGN, but this classification is becoming obsolete as the causes of this pattern are becoming understood.[citation needed]

Type I

Type I, the most common by far, is caused by immune complexes depositing in the kidney. It is characterised by subendothelial and mesangial immune deposits.[citation needed]

It is believed to be associated with the classical complement pathway.[4]

Type II

See also: Eculizumab § Dense-deposit disease (DDD)

Also called recently as ‘C3 nephropathy’ The preferred name is "dense deposit disease."[5] Most cases of dense deposit disease do not show a membranoproliferative pattern.[6] A 2012 review considers DDD to be in a continuum with C3 glomerulonephritis,[7] one reason the use of the type I to type III classification system is falling out of favour.[citation needed]

Most cases are associated with the dysregulation of the alternative complement pathway.[8][9]

DDD is associated with deposition of complement C3 within the glomeruli with little or no staining for immunoglobulin. The presence of C3 without significant immunoglobulin suggested to early investigators that DDD was due to abnormal activation of the complement alternative pathway (AP). There is now strong evidence that DDD is caused by uncontrolled AP activation.[10]

Spontaneous remissions of MPGN II are rare; approximately half of those affected with MPGN II will progress to end stage renal disease within ten years.[11]

In many cases, people with MPGN II can develop drusen caused by deposits within Bruch's membrane beneath the retinal pigment epithelium of the eye. Over time, vision can deteriorate, and subretinal neovascular membranes, macular detachment, and central serous retinopathy can develop.[12]

Type III

Type III is very rare, it is characterized by a mixture of subepithelial and subendothelial immune and/or complement deposits. These deposits elicit an immune response, causing damage to cells and structures within their vicinity. Has similar pathological findings of Type I disease.[13]

A candidate gene has been identified on chromosome 1.[14]

Complement component 3 is seen under immunofluorescence.[15] it is associated with complement receptor 6 deficiency.

Pathology

 
Renal corpuscle. Membranoproliferative glomerulonephritis involves deposits at the intraglomerular mesangium which leads to "splitting" of the glomerular basement membrane.

Membranoproliferative glomerulonephritis involves deposits at the intraglomerular mesangium.[citation needed]

It is also the main hepatitis C associated nephropathy.[citation needed]

It also is related to a number of autoimmune diseases, prominently systemic lupus erythematosus (SLE), Class IV. Also found with Sjögren syndrome, rheumatoid arthritis, inherited complement deficiencies (esp C3 deficiency), scleroderma, Celiac disease.[16]

The histomorphologic differential diagnosis includes transplant glomerulopathy and thrombotic microangiopathies.[citation needed]

Diagnosis

The GBM is rebuilt on top of the deposits, causing a "tram tracking" appearance under the microscope.[17] Mesangial cellularity is increased.[18]

Treatment

Primary MPGN is treated with steroids, plasma exchange and other immunosuppressive drugs. Secondary MPGN is treated by treating the associated infection, autoimmune disease or neoplasms. Pegylated interferon and ribavirin are useful in reducing viral load. [19]

See also

References

  1. ^ Colville D, Guymer R, Sinclair RA, Savige J (August 2003). "Visual impairment caused by retinal abnormalities in mesangiocapillary (membranoproliferative) glomerulonephritis type II ("dense deposit disease")". Am. J. Kidney Dis. 42 (2): E2–5. doi:10.1016/S0272-6386(03)00665-6. PMID 12900843.
  2. ^ "" at Dorland's Medical Dictionary
  3. ^ Habib R, Gubler MC, Loirat C, Mäiz HB, Levy M (1975). "Dense deposit disease: a variant of membranoproliferative glomerulonephritis". Kidney Int. 7 (4): 204–15. doi:10.1038/ki.1975.32. PMID 1095806.
  4. ^ West CD, McAdams AJ (March 1998). "Glomerular paramesangial deposits: association with hypocomplementemia in membranoproliferative glomerulonephritis types I and III". Am. J. Kidney Dis. 31 (3): 427–34. doi:10.1053/ajkd.1998.v31.pm9506679. PMID 9506679.
  5. ^ "Final Diagnosis — Case 148". Retrieved 2008-11-25.
  6. ^ Patrick D Walker; Franco Ferrario; Kensuke Joh; Stephen M Bonsib (2007). "Dense deposit disease is not a membranoproliferative glomerulonephritis". Modern Pathology. 20 (6): 605–616. doi:10.1038/modpathol.3800773. PMID 17396142.
  7. ^ Sethi S, Fervenza FC (2012). "Membranoproliferative glomerulonephritis - a new look at an old entity". N Engl J Med. 366 (12): 1119–1131. doi:10.1056/NEJMra1108178. PMID 22435371.
  8. ^ Rose KL, Paixao-Cavalcante D, Fish J, et al. (February 2008). "Factor I is required for the development of membranoproliferative glomerulonephritis in factor H-deficient mice". J. Clin. Invest. 118 (2): 608–18. doi:10.1172/JCI32525. PMC 2200299. PMID 18202746.
  9. ^ Licht C, Schlötzer-Schrehardt U, Kirschfink M, Zipfel PF, Hoppe B (January 2007). "MPGN II--genetically determined by defective complement regulation?". Pediatr. Nephrol. 22 (1): 2–9. doi:10.1007/s00467-006-0299-8. PMID 17024390. S2CID 14776253.
  10. ^ (reviewed in Appel et al., 2005; Smith et al., 2007). Smith, R. J. ., Harris, C. L., & Pickering, M. C. (2011). Dense Deposit Disease. Molecular Immunology, 48(14), 1604–1610. http://doi.org/10.1016/j.molimm.2011.04.005/
  11. ^ Swainson CP, Robson JS, Thomson D, MacDonald MK (1983). "Mesangiocapillary glomerulonephritis: a long-term study of 40 cases". J. Pathol. 141 (4): 449–68. doi:10.1002/path.1711410404. PMID 6363655. S2CID 25434508.
  12. ^ Colville D, Guymer R, Sinclair RA, Savige J (2003). "Visual impairment caused by retinal abnormalities in mesangiocapillary (membranoproliferative) glomerulonephritis type II ("dense deposit disease")". Am. J. Kidney Dis. 42 (2): E2–5. doi:10.1016/S0272-6386(03)00665-6. PMID 12900843.
  13. ^ Pickering, M. C., D’Agati, V. D., Nester, C. M., Smith, R. J., Haas, M., Appel, G. B., … Cook, H. T. (2013). C3 glomerulopathy: consensus report. Kidney International, 84(6), 1079–1089. http://doi.org/10.1038/ki.2013.377
  14. ^ Neary JJ, Conlon PJ, Croke D, et al. (August 2002). "Linkage of a gene causing familial membranoproliferative glomerulonephritis type III to chromosome 1". J. Am. Soc. Nephrol. 13 (8): 2052–7. doi:10.1097/01.ASN.0000022006.49966.F8. PMID 12138136.
  15. ^ Neary J, Dorman A, Campbell E, Keogan M, Conlon P (July 2002). "Familial membranoproliferative glomerulonephritis type III". Am. J. Kidney Dis. 40 (1): e1.1–e1.6. doi:10.1053/ajkd.2002.33932. PMID 12087587.
  16. ^ "UpToDate".
  17. ^ . Archived from the original on 2006-09-10. Retrieved 2008-11-25.
  18. ^ "Renal Pathology". Retrieved 2008-11-25.
  19. ^ Harrison's principles of internal medicine (19th ed.). New York, NY: McGraw-Hill Companies, Inc. 2015. p. 1841. ISBN 978-0-07-180216-1.

External links

  • Glomerulonephritis, Membranoproliferative Types I, II, III at eMedicine
  • Corchado, Johnny Cruz, Smith, Richard JH (July 2007). "Dense Deposit Disease/Membranoproliferative Glomerulonephritis Type II". In Pagon RA, Bird TD, Dolan CR, et al. (eds.). GeneReviews. Seattle WA: University of Washington. PMID 20301598.
  • Membranoproliferative_GN at Nephropathology tutorial
  • MP GN Pathophysiology discusses the nephritic auto-antibodies/factors

March 04, 2023
membranoproliferative, glomerulonephritis, mpgn, type, glomerulonephritis, caused, deposits, kidney, glomerular, mesangium, basement, membrane, thickening, activating, complement, damaging, glomeruli, other, namesmesangiocapillary, glomerulonephritis, microgra. Membranoproliferative glomerulonephritis MPGN is a type of glomerulonephritis caused by deposits in the kidney glomerular mesangium and basement membrane GBM thickening 2 activating complement and damaging the glomeruli Membranoproliferative glomerulonephritisOther namesMesangiocapillary glomerulonephritis 1 Micrograph of glomerulus in membranoproliferative glomerulonephritis with increased mesangial matrix and increased mesangial cellularity Kidney biopsy PAS stain SpecialtyNephrologyMPGN accounts for approximately 4 of primary renal causes of nephrotic syndrome in children and 7 in adults 3 It should not be confused with membranous glomerulonephritis a condition in which the basement membrane is thickened but the mesangium is not Contents 1 Type 1 1 Type I 1 2 Type II 1 3 Type III 2 Pathology 3 Diagnosis 4 Treatment 5 See also 6 References 7 External linksType EditThere are three types of MPGN but this classification is becoming obsolete as the causes of this pattern are becoming understood citation needed Type I Edit Type I the most common by far is caused by immune complexes depositing in the kidney It is characterised by subendothelial and mesangial immune deposits citation needed It is believed to be associated with the classical complement pathway 4 Type II Edit See also Eculizumab Dense deposit disease DDD Also called recently as C3 nephropathy The preferred name is dense deposit disease 5 Most cases of dense deposit disease do not show a membranoproliferative pattern 6 A 2012 review considers DDD to be in a continuum with C3 glomerulonephritis 7 one reason the use of the type I to type III classification system is falling out of favour citation needed Most cases are associated with the dysregulation of the alternative complement pathway 8 9 DDD is associated with deposition of complement C3 within the glomeruli with little or no staining for immunoglobulin The presence of C3 without significant immunoglobulin suggested to early investigators that DDD was due to abnormal activation of the complement alternative pathway AP There is now strong evidence that DDD is caused by uncontrolled AP activation 10 Spontaneous remissions of MPGN II are rare approximately half of those affected with MPGN II will progress to end stage renal disease within ten years 11 In many cases people with MPGN II can develop drusen caused by deposits within Bruch s membrane beneath the retinal pigment epithelium of the eye Over time vision can deteriorate and subretinal neovascular membranes macular detachment and central serous retinopathy can develop 12 Type III Edit Type III is very rare it is characterized by a mixture of subepithelial and subendothelial immune and or complement deposits These deposits elicit an immune response causing damage to cells and structures within their vicinity Has similar pathological findings of Type I disease 13 A candidate gene has been identified on chromosome 1 14 Complement component 3 is seen under immunofluorescence 15 it is associated with complement receptor 6 deficiency Pathology Edit Renal corpuscle Membranoproliferative glomerulonephritis involves deposits at the intraglomerular mesangium which leads to splitting of the glomerular basement membrane Membranoproliferative glomerulonephritis involves deposits at the intraglomerular mesangium citation needed It is also the main hepatitis C associated nephropathy citation needed It also is related to a number of autoimmune diseases prominently systemic lupus erythematosus SLE Class IV Also found with Sjogren syndrome rheumatoid arthritis inherited complement deficiencies esp C3 deficiency scleroderma Celiac disease 16 The histomorphologic differential diagnosis includes transplant glomerulopathy and thrombotic microangiopathies citation needed Diagnosis EditThe GBM is rebuilt on top of the deposits causing a tram tracking appearance under the microscope 17 Mesangial cellularity is increased 18 Treatment EditPrimary MPGN is treated with steroids plasma exchange and other immunosuppressive drugs Secondary MPGN is treated by treating the associated infection autoimmune disease or neoplasms Pegylated interferon and ribavirin are useful in reducing viral load 19 See also EditDiffuse proliferative nephritisReferences Edit Colville D Guymer R Sinclair RA Savige J August 2003 Visual impairment caused by retinal abnormalities in mesangiocapillary membranoproliferative glomerulonephritis type II dense deposit disease Am J Kidney Dis 42 2 E2 5 doi 10 1016 S0272 6386 03 00665 6 PMID 12900843 membranoproliferative glomerulonephritis at Dorland s Medical Dictionary Habib R Gubler MC Loirat C Maiz HB Levy M 1975 Dense deposit disease a variant of membranoproliferative glomerulonephritis Kidney Int 7 4 204 15 doi 10 1038 ki 1975 32 PMID 1095806 West CD McAdams AJ March 1998 Glomerular paramesangial deposits association with hypocomplementemia in membranoproliferative glomerulonephritis types I and III Am J Kidney Dis 31 3 427 34 doi 10 1053 ajkd 1998 v31 pm9506679 PMID 9506679 Final Diagnosis Case 148 Retrieved 2008 11 25 Patrick D Walker Franco Ferrario Kensuke Joh Stephen M Bonsib 2007 Dense deposit disease is not a membranoproliferative glomerulonephritis Modern Pathology 20 6 605 616 doi 10 1038 modpathol 3800773 PMID 17396142 Sethi S Fervenza FC 2012 Membranoproliferative glomerulonephritis a new look at an old entity N Engl J Med 366 12 1119 1131 doi 10 1056 NEJMra1108178 PMID 22435371 Rose KL Paixao Cavalcante D Fish J et al February 2008 Factor I is required for the development of membranoproliferative glomerulonephritis in factor H deficient mice J Clin Invest 118 2 608 18 doi 10 1172 JCI32525 PMC 2200299 PMID 18202746 Licht C Schlotzer Schrehardt U Kirschfink M Zipfel PF Hoppe B January 2007 MPGN II genetically determined by defective complement regulation Pediatr Nephrol 22 1 2 9 doi 10 1007 s00467 006 0299 8 PMID 17024390 S2CID 14776253 reviewed in Appel et al 2005 Smith et al 2007 Smith R J Harris C L amp Pickering M C 2011 Dense Deposit Disease Molecular Immunology 48 14 1604 1610 http doi org 10 1016 j molimm 2011 04 005 Swainson CP Robson JS Thomson D MacDonald MK 1983 Mesangiocapillary glomerulonephritis a long term study of 40 cases J Pathol 141 4 449 68 doi 10 1002 path 1711410404 PMID 6363655 S2CID 25434508 Colville D Guymer R Sinclair RA Savige J 2003 Visual impairment caused by retinal abnormalities in mesangiocapillary membranoproliferative glomerulonephritis type II dense deposit disease Am J Kidney Dis 42 2 E2 5 doi 10 1016 S0272 6386 03 00665 6 PMID 12900843 Pickering M C D Agati V D Nester C M Smith R J Haas M Appel G B Cook H T 2013 C3 glomerulopathy consensus report Kidney International 84 6 1079 1089 http doi org 10 1038 ki 2013 377 Neary JJ Conlon PJ Croke D et al August 2002 Linkage of a gene causing familial membranoproliferative glomerulonephritis type III to chromosome 1 J Am Soc Nephrol 13 8 2052 7 doi 10 1097 01 ASN 0000022006 49966 F8 PMID 12138136 Neary J Dorman A Campbell E Keogan M Conlon P July 2002 Familial membranoproliferative glomerulonephritis type III Am J Kidney Dis 40 1 e1 1 e1 6 doi 10 1053 ajkd 2002 33932 PMID 12087587 UpToDate Membranoproliferative glomerulonephritis type I of the Kidney Archived from the original on 2006 09 10 Retrieved 2008 11 25 Renal Pathology Retrieved 2008 11 25 Harrison s principles of internal medicine 19th ed New York NY McGraw Hill Companies Inc 2015 p 1841 ISBN 978 0 07 180216 1 External links EditGlomerulonephritis Membranoproliferative Types I II III at eMedicine Corchado Johnny Cruz Smith Richard JH July 2007 Dense Deposit Disease Membranoproliferative Glomerulonephritis Type II In Pagon RA Bird TD Dolan CR et al eds GeneReviews Seattle WA University of Washington PMID 20301598 Membranoproliferative GN at Nephropathology tutorial MP GN Pathophysiology discusses the nephritic auto antibodies factors Retrieved from https en wikipedia org w index php title Membranoproliferative glomerulonephritis amp oldid 1124004671, wikipedia, wiki, book, books, library,

article

, read, download, free, free download, mp3, video, mp4, 3gp, jpg, jpeg, gif, png, picture, music, song, movie, book, game, games.