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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

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis.
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
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis.
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
ANCA-associated vasculitis: diagnosis, clinical characteristics and treatment.
Haubitz M
Vasa 2007 May;36(2):81-9. doi: 10.1024/0301-1526.36.2.81. PMID: 17708098

Recent clinical studies

Etiology

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis.
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
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis.
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
ANCA Glomerulonephritis and Vasculitis.
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
ANCA-associated pauci-immune glomerulonephritis: always pauci-immune?
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
Rituximab versus cyclophosphamide for ANCA-associated vasculitis with renal involvement.
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

Diagnosis

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis.
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
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis.
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
ANCA Glomerulonephritis and Vasculitis.
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
ANCA-associated pauci-immune glomerulonephritis: always pauci-immune?
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
Pauci-immune necrotizing glomerulonephritis.
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

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).
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
ANCA Glomerulonephritis and Vasculitis.
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
Rituximab versus cyclophosphamide for ANCA-associated vasculitis with renal involvement.
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
Pauci-immune necrotizing glomerulonephritis.
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
Silicon exposure and vasculitis.
Tervaert JW, Stegeman CA, Kallenberg CG
Curr Opin Rheumatol 1998 Jan;10(1):12-7. doi: 10.1097/00002281-199801000-00003. PMID: 9448985

Prognosis

Renal Involvement in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
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
Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis.
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
Histopathological classification of pauci-immune glomerulonephritis and its impact on outcome.
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
Renal involvement in anti-neutrophil cytoplasmic autoantibody associated vasculitis.
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
Pauci-immune necrotizing glomerulonephritis.
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

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis.
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
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis.
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
Rituximab versus cyclophosphamide for ANCA-associated vasculitis with renal involvement.
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
Histopathological classification of pauci-immune glomerulonephritis and its impact on outcome.
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
Renal involvement in anti-neutrophil cytoplasmic autoantibody associated vasculitis.
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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