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Pauci-Immune Glomerulonephritis associated with Granulomatosis with Polyangiitis

MedGen UID:
887463
Concept ID:
C4050407
Disease or Syndrome

Definition

Glomerulonephritis in the context of granulomatosis with polyangiitis in which anti-neutrophil cytoplasm antibody (ANCA) is almost always present. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPauci-Immune Glomerulonephritis associated with Granulomatosis with Polyangiitis

Professional guidelines

PubMed

Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, Khalid S, Hutchings A, Watts RA, Merkel PA, Luqmani RA; DCVAS Investigators
Ann Rheum Dis 2022 Mar;81(3):315-320. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221795. PMID: 35110333
Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, Khalid S, Hutchings A, Watts RA, Merkel PA, Luqmani RA; DCVAS Study Group
Arthritis Rheumatol 2022 Mar;74(3):393-399. Epub 2022 Feb 2 doi: 10.1002/art.41986. PMID: 35106964
Haubitz M
Vasa 2007 May;36(2):81-9. doi: 10.1024/0301-1526.36.2.81. PMID: 17708098

Recent clinical studies

Etiology

Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, Khalid S, Hutchings A, Watts RA, Merkel PA, Luqmani RA; DCVAS Investigators
Ann Rheum Dis 2022 Mar;81(3):315-320. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221795. PMID: 35110333
Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, Khalid S, Hutchings A, Watts RA, Merkel PA, Luqmani RA; DCVAS Study Group
Arthritis Rheumatol 2022 Mar;74(3):393-399. Epub 2022 Feb 2 doi: 10.1002/art.41986. PMID: 35106964
Venade G, Figueiredo C, Almeida C, Oliveira N, Matos LC
Rev Assoc Med Bras (1992) 2020 Jul;66(7):904-907. Epub 2020 Aug 24 doi: 10.1590/1806-9282.66.7.904. PMID: 32844923
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
Scaglioni V, Scolnik M, Catoggio LJ, Christiansen SB, Varela CF, Greloni G, Rosa-Diez G, Soriano ER
Clin Exp Rheumatol 2017 Mar-Apr;35 Suppl 103(1):55-58. Epub 2017 Jan 31 PMID: 28229825

Diagnosis

Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, Khalid S, Hutchings A, Watts RA, Merkel PA, Luqmani RA; DCVAS Investigators
Ann Rheum Dis 2022 Mar;81(3):315-320. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221795. PMID: 35110333
Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, Khalid S, Hutchings A, Watts RA, Merkel PA, Luqmani RA; DCVAS Study Group
Arthritis Rheumatol 2022 Mar;74(3):393-399. Epub 2022 Feb 2 doi: 10.1002/art.41986. PMID: 35106964
Guzman-Soto MI, Kimura Y, Romero-Sanchez G, Cienfuegos-Alvear JA, Candanedo-Gonzalez F, Kimura-Sandoval Y, Sanchez-Nava DA, Alonso-Ramon I, Hinojosa-Azaola A
Radiographics 2021 Nov-Dec;41(7):1973-1991. Epub 2021 Oct 15 doi: 10.1148/rg.2021210132. PMID: 34652975
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
Rutgers A, Sanders JS, Stegeman CA, Kallenberg CG
Rheum Dis Clin North Am 2010 Aug;36(3):559-72. doi: 10.1016/j.rdc.2010.05.002. PMID: 20688250

Therapy

Venade G, Figueiredo C, Almeida C, Oliveira N, Matos LC
Rev Assoc Med Bras (1992) 2020 Jul;66(7):904-907. Epub 2020 Aug 24 doi: 10.1590/1806-9282.66.7.904. PMID: 32844923
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
Geetha D, Specks U, Stone JH, Merkel PA, Seo P, Spiera R, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Fessler BJ, Ding L, Tchao NK, Ikle D, Jepson B, Brunetta P, Fervenza FC; Rituximab for ANCA-Associated Vasculitis Immune Tolerance Network Research Group
J Am Soc Nephrol 2015 Apr;26(4):976-85. Epub 2014 Nov 7 doi: 10.1681/ASN.2014010046. PMID: 25381429Free PMC Article
Rutgers A, Sanders JS, Stegeman CA, Kallenberg CG
Rheum Dis Clin North Am 2010 Aug;36(3):559-72. doi: 10.1016/j.rdc.2010.05.002. PMID: 20688250
Tervaert JW, Stegeman CA, Kallenberg CG
Curr Opin Rheumatol 1998 Jan;10(1):12-7. doi: 10.1097/00002281-199801000-00003. PMID: 9448985

Prognosis

Zonozi R, Niles JL, Cortazar FB
Rheum Dis Clin North Am 2018 Nov;44(4):525-543. Epub 2018 Sep 7 doi: 10.1016/j.rdc.2018.06.001. PMID: 30274621
Syed R, Rehman A, Valecha G, El-Sayegh S
Biomed Res Int 2015;2015:402826. Epub 2015 Nov 25 doi: 10.1155/2015/402826. PMID: 26688808Free PMC Article
Naidu GS, Sharma A, Nada R, Kohli HS, Jha V, Gupta KL, Sakhuja V, Rathi M
Rheumatol Int 2014 Dec;34(12):1721-7. Epub 2014 May 18 doi: 10.1007/s00296-014-3041-z. PMID: 24838685
Sinico RA, Di Toma L, Radice A
Autoimmun Rev 2013 Feb;12(4):477-82. Epub 2012 Aug 16 doi: 10.1016/j.autrev.2012.08.006. PMID: 22921791
Rutgers A, Sanders JS, Stegeman CA, Kallenberg CG
Rheum Dis Clin North Am 2010 Aug;36(3):559-72. doi: 10.1016/j.rdc.2010.05.002. PMID: 20688250

Clinical prediction guides

Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, Khalid S, Hutchings A, Watts RA, Merkel PA, Luqmani RA; DCVAS Investigators
Ann Rheum Dis 2022 Mar;81(3):315-320. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221795. PMID: 35110333
Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, Khalid S, Hutchings A, Watts RA, Merkel PA, Luqmani RA; DCVAS Study Group
Arthritis Rheumatol 2022 Mar;74(3):393-399. Epub 2022 Feb 2 doi: 10.1002/art.41986. PMID: 35106964
Geetha D, Specks U, Stone JH, Merkel PA, Seo P, Spiera R, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Fessler BJ, Ding L, Tchao NK, Ikle D, Jepson B, Brunetta P, Fervenza FC; Rituximab for ANCA-Associated Vasculitis Immune Tolerance Network Research Group
J Am Soc Nephrol 2015 Apr;26(4):976-85. Epub 2014 Nov 7 doi: 10.1681/ASN.2014010046. PMID: 25381429Free PMC Article
Naidu GS, Sharma A, Nada R, Kohli HS, Jha V, Gupta KL, Sakhuja V, Rathi M
Rheumatol Int 2014 Dec;34(12):1721-7. Epub 2014 May 18 doi: 10.1007/s00296-014-3041-z. PMID: 24838685
Sinico RA, Di Toma L, Radice A
Autoimmun Rev 2013 Feb;12(4):477-82. Epub 2012 Aug 16 doi: 10.1016/j.autrev.2012.08.006. PMID: 22921791

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