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Pauci-Immune Glomerulonephritis associated with Eosinophilic Granulomatosis with Polyangiitis

MedGen UID:
881382
•Concept ID:
C4054225
•
Disease or Syndrome

Definition

Glomerulonephritis in the context of eosinophilic-rich granulomatosis with polyangiitis, eosinophilia, asthma and commonly anti-neutrophil cytoplasmic antibody. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood.
Cabral DA, Uribe AG, Benseler S, O'Neil KM, Hashkes PJ, Higgins G, Zeft AS, Lovell DJ, Kingsbury DJ, Stevens A, McCurdy D, Chira P, Abramson L, Arkachaisri T, Campillo S, Eberhard A, Hersh AO, Huber AM, Kim S, Klein-Gitelman M, Levy DM, Li SC, Mason T, Dewitt EM, Muscal E, Nassi L, Reiff A, Schikler K, Singer NG, Wahezi D, Woodward A; ARChiVe (A Registry for Childhood Vasculitis: e-entry) Investigators Network
Arthritis Rheum 2009 Nov;60(11):3413-24. doi: 10.1002/art.24876. PMID: 19877069
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
Addendum to the International Consensus Statement on testing and reporting of antineutrophil cytoplasmic antibodies. Quality control guidelines, comments, and recommendations for testing in other autoimmune diseases.
Savige J, Dimech W, Fritzler M, Goeken J, Hagen EC, Jennette JC, McEvoy R, Pusey C, Pollock W, Trevisin M, Wiik A, Wong R; International Group for Consensus Statement on Testing and Reporting of Antineutrophil Cytoplasmic Antibodies (ANCA)
Am J Clin Pathol 2003 Sep;120(3):312-8. doi: 10.1309/WAEP-ADW0-K4LP-UHFN. PMID: 14502794

Recent clinical studies

Etiology

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
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
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
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

Diagnosis

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
Antineutrophil cytoplasmic antibodies.
Bosch X, Guilabert A, Font J
Lancet 2006 Jul 29;368(9533):404-18. doi: 10.1016/S0140-6736(06)69114-9. PMID: 16876669
Pauci-immune crescentic glomerulonephritis.
Gupta RK
Indian J Pathol Microbiol 2003 Jul;46(3):357-66. PMID: 15025274

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
An overlap of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis.
Surendran S, Gundappa C, Gandhi A, Kurien AA, Fernando E
Saudi J Kidney Dis Transpl 2017 May-Jun;28(3):639-644. doi: 10.4103/1319-2442.206450. PMID: 28540906
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
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

Prognosis

Simultaneous development of IgA vasculitis and eosinophilic granulomatosis with polyangiitis.
Asano Y, Matsumoto Y, Miyazaki T, Ishizu A, Morizane S, Hayashi K, Yamamura Y, Hiramatsu S, Miyawaki Y, Morishita M, Ohashi K, Watanabe H, Watanabe KS, Kawabata T, Sada KE, Makino H, Wada J
Mod Rheumatol Case Rep 2020 Jan;4(1):63-69. Epub 2019 Oct 23 doi: 10.1080/24725625.2019.1673528. PMID: 33086980
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
Pauci-immune crescentic glomerulonephritis.
Gupta RK
Indian J Pathol Microbiol 2003 Jul;46(3):357-66. PMID: 15025274

Clinical prediction guides

Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
Morishita KA, Moorthy LN, Lubieniecka JM, Twilt M, Yeung RSM, Toth MB, Shenoi S, Ristic G, Nielsen SM, Luqmani RA, Li SC, Lee T, Lawson EF, Kostik MM, Klein-Gitelman M, Huber AM, Hersh AO, Foell D, Elder ME, Eberhard BA, Dancey P, Charuvanij S, Benseler SM, Cabral DA; ARChiVe Investigators Network within the PedVas Initiative
Arthritis Rheumatol 2017 Jul;69(7):1470-1479. Epub 2017 Jun 9 doi: 10.1002/art.40112. PMID: 28371513
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
The role of myeloperoxidase in the pathogenesis of systemic vasculitis.
Rutgers A, Heeringa P, Tervaert JW
Clin Exp Rheumatol 2003 Nov-Dec;21(6 Suppl 32):S55-63. PMID: 14740428
Clinical spectrum associated with ANCA of defined antigen specificities in 98 selected patients.
Geffriaud-Ricouard C, Noël LH, Chauveau D, Houhou S, Grünfeld JP, Lesavre P
Clin Nephrol 1993 Mar;39(3):125-36. PMID: 7681736

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