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Pauci-Immune Glomerulonephritis - ANCA Negative

MedGen UID:
890175
Concept ID:
C4054226
Disease or Syndrome

Definition

Glomerulonephritis with paucity of glomerular staining for immunoglobulins that may be accompanied by systemic, small vessel vasculitis containing no anti neutrophil cytoplasm antibody (ANCA). [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPauci-Immune Glomerulonephritis - ANCA Negative

Professional guidelines

PubMed

Hruskova Z, Tesar V
Blood Purif 2018;45(1-3):213-217. Epub 2018 Jan 26 doi: 10.1159/000485368. PMID: 29478046
Damoiseaux J, Vaessen M, Knapen Y, Csernok E, Stegeman CA, Van Paassen P, Tervaert JW
Ann N Y Acad Sci 2007 Aug;1109:454-63. doi: 10.1196/annals.1398.051. PMID: 17785334
Kitching AR, Hutchinson P, Atkins RC, Holdsworth SR
Nephrol Dial Transplant 2004 Feb;19(2):365-70. doi: 10.1093/ndt/gfg553. PMID: 14736960

Recent clinical studies

Etiology

Ronsin C, Georges M, Chapelet-Debout A, Augusto JF, Audard V, Lebourg L, Rubin S, Quemeneur T, Bataille P, Karras A, Daugas E, Titeca-Beauport D, Boffa JJ, Vigneau C, Halimi JM, Isnard-Bagnis C, Durault S, Renaudineau E, Bridoux F, Testa A, Le Quintrec M, Renaudin K, Fakhouri F
Am J Kidney Dis 2022 Jan;79(1):56-68.e1. Epub 2021 Jun 11 doi: 10.1053/j.ajkd.2021.03.027. PMID: 34119564
Buglioni A, Fidler ME, Alexander MP, Sethi S, Nasr SH, Hernandez LPH, Grande JP, Cosio FG, Cornell LD
Mod Pathol 2020 Mar;33(3):440-447. Epub 2019 Sep 2 doi: 10.1038/s41379-019-0355-0. PMID: 31477812
Jennette JC, Nachman PH
Clin J Am Soc Nephrol 2017 Oct 6;12(10):1680-1691. Epub 2017 Aug 25 doi: 10.2215/CJN.02500317. PMID: 28842398Free PMC Article
Hilhorst M, van Paassen P, van Rie H, Bijnens N, Heerings-Rewinkel P, van Breda Vriesman P, Cohen Tervaert JW; Limburg Renal Registry
Nephrol Dial Transplant 2017 Aug 1;32(8):1302-1313. doi: 10.1093/ndt/gfv288. PMID: 26275893
Villacorta J, Diaz-Crespo F, Acevedo M, Guerrero C, Mollejo M, Fernandez-Juarez G
Nephrology (Carlton) 2016 Apr;21(4):301-7. doi: 10.1111/nep.12608. PMID: 26369898

Diagnosis

Ronsin C, Georges M, Chapelet-Debout A, Augusto JF, Audard V, Lebourg L, Rubin S, Quemeneur T, Bataille P, Karras A, Daugas E, Titeca-Beauport D, Boffa JJ, Vigneau C, Halimi JM, Isnard-Bagnis C, Durault S, Renaudineau E, Bridoux F, Testa A, Le Quintrec M, Renaudin K, Fakhouri F
Am J Kidney Dis 2022 Jan;79(1):56-68.e1. Epub 2021 Jun 11 doi: 10.1053/j.ajkd.2021.03.027. PMID: 34119564
Buglioni A, Fidler ME, Alexander MP, Sethi S, Nasr SH, Hernandez LPH, Grande JP, Cosio FG, Cornell LD
Mod Pathol 2020 Mar;33(3):440-447. Epub 2019 Sep 2 doi: 10.1038/s41379-019-0355-0. PMID: 31477812
Oxley Oxland J, Ensor J, Freercks R
BMJ Case Rep 2018 Feb 5;2018 doi: 10.1136/bcr-2017-221948. PMID: 29437730Free PMC Article
Jennette JC, Nachman PH
Clin J Am Soc Nephrol 2017 Oct 6;12(10):1680-1691. Epub 2017 Aug 25 doi: 10.2215/CJN.02500317. PMID: 28842398Free PMC Article
Villacorta J, Diaz-Crespo F, Acevedo M, Guerrero C, Mollejo M, Fernandez-Juarez G
Nephrology (Carlton) 2016 Apr;21(4):301-7. doi: 10.1111/nep.12608. PMID: 26369898

Therapy

Ronsin C, Georges M, Chapelet-Debout A, Augusto JF, Audard V, Lebourg L, Rubin S, Quemeneur T, Bataille P, Karras A, Daugas E, Titeca-Beauport D, Boffa JJ, Vigneau C, Halimi JM, Isnard-Bagnis C, Durault S, Renaudineau E, Bridoux F, Testa A, Le Quintrec M, Renaudin K, Fakhouri F
Am J Kidney Dis 2022 Jan;79(1):56-68.e1. Epub 2021 Jun 11 doi: 10.1053/j.ajkd.2021.03.027. PMID: 34119564
Buglioni A, Fidler ME, Alexander MP, Sethi S, Nasr SH, Hernandez LPH, Grande JP, Cosio FG, Cornell LD
Mod Pathol 2020 Mar;33(3):440-447. Epub 2019 Sep 2 doi: 10.1038/s41379-019-0355-0. PMID: 31477812
Oxley Oxland J, Ensor J, Freercks R
BMJ Case Rep 2018 Feb 5;2018 doi: 10.1136/bcr-2017-221948. PMID: 29437730Free PMC Article
Jennette JC, Nachman PH
Clin J Am Soc Nephrol 2017 Oct 6;12(10):1680-1691. Epub 2017 Aug 25 doi: 10.2215/CJN.02500317. PMID: 28842398Free PMC Article
Villacorta J, Diaz-Crespo F, Acevedo M, Guerrero C, Mollejo M, Fernandez-Juarez G
Nephrology (Carlton) 2016 Apr;21(4):301-7. doi: 10.1111/nep.12608. PMID: 26369898

Prognosis

Gallan AJ, Alexander E, Reid P, Kutuby F, Chang A, Henriksen KJ
Am J Kidney Dis 2019 Dec;74(6):853-856. Epub 2019 Jun 14 doi: 10.1053/j.ajkd.2019.04.016. PMID: 31204194
Oxley Oxland J, Ensor J, Freercks R
BMJ Case Rep 2018 Feb 5;2018 doi: 10.1136/bcr-2017-221948. PMID: 29437730Free PMC Article
Villacorta J, Diaz-Crespo F, Acevedo M, Guerrero C, Mollejo M, Fernandez-Juarez G
Nephrology (Carlton) 2016 Apr;21(4):301-7. doi: 10.1111/nep.12608. PMID: 26369898
Sharma A, Nada R, Naidu GS, Minz RW, Kohli HS, Sakhuja V, Gupta KL, Rathi M
Int J Rheum Dis 2016 Jan;19(1):74-81. Epub 2015 Aug 13 doi: 10.1111/1756-185X.12729. PMID: 26269223
Barrett CM, Troxell ML, Larsen CP, Houghton DC
Int Urol Nephrol 2014 May;46(5):963-71. Epub 2013 Nov 12 doi: 10.1007/s11255-013-0593-x. PMID: 24217802

Clinical prediction guides

Ronsin C, Georges M, Chapelet-Debout A, Augusto JF, Audard V, Lebourg L, Rubin S, Quemeneur T, Bataille P, Karras A, Daugas E, Titeca-Beauport D, Boffa JJ, Vigneau C, Halimi JM, Isnard-Bagnis C, Durault S, Renaudineau E, Bridoux F, Testa A, Le Quintrec M, Renaudin K, Fakhouri F
Am J Kidney Dis 2022 Jan;79(1):56-68.e1. Epub 2021 Jun 11 doi: 10.1053/j.ajkd.2021.03.027. PMID: 34119564
Rodríguez E, Latzke B, Sierra M, Romera AM, Siedel D, Agraz I, Soler MJ, García-Carro C, Draibe J, de la Prada FJ, Villacorta J, Buxeda A, Sierra-Ochoa A, Lozano I, Durán X, Barrios C, Pascual J
Nephrol Dial Transplant 2022 Mar 25;37(4):697-704. doi: 10.1093/ndt/gfab020. PMID: 33533909
Villacorta J, Diaz-Crespo F, Acevedo M, Guerrero C, Mollejo M, Fernandez-Juarez G
Nephrology (Carlton) 2016 Apr;21(4):301-7. doi: 10.1111/nep.12608. PMID: 26369898
Sharma A, Nada R, Naidu GS, Minz RW, Kohli HS, Sakhuja V, Gupta KL, Rathi M
Int J Rheum Dis 2016 Jan;19(1):74-81. Epub 2015 Aug 13 doi: 10.1111/1756-185X.12729. PMID: 26269223
Eisenberger U, Fakhouri F, Vanhille P, Beaufils H, Mahr A, Guillevin L, Lesavre P, Noël LH
Nephrol Dial Transplant 2005 Jul;20(7):1392-9. Epub 2005 Apr 26 doi: 10.1093/ndt/gfh830. PMID: 15855209

Recent systematic reviews

Floyd L, Morris AD, Elsayed ME, Shetty A, Baksi A, Geetha D, Dhaygude A, Mitra S
Kidney360 2023 Jan 1;4(1):69-77. Epub 2022 Nov 24 doi: 10.34067/KID.0003892022. PMID: 36700906Free PMC Article
Van Gool IC, Kers J, Bakker JA, Rotmans JI, Teng YKO, Bauer MP
Clin Rheumatol 2022 Oct;41(10):2949-2960. Epub 2022 Jun 23 doi: 10.1007/s10067-022-06240-w. PMID: 35732985Free PMC Article

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