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Pauci-Immune Glomerulonephritis - Renal Limited

MedGen UID:
886335
•Concept ID:
C4053792
•
Disease or Syndrome

Definition

Glomerulonephritis in which anti-neutrophil cytoplasm antibody (ANCA) is almost always present and vasculitis is limited to the kidney. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPauci-Immune Glomerulonephritis - Renal Limited

Professional guidelines

PubMed

Management of Elderly Patients with Rapidly Progressive Glomerulonephritis.
Hruskova Z, Tesar V
Blood Purif 2018;45(1-3):213-217. Epub 2018 Jan 26 doi: 10.1159/000485368. PMID: 29478046
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

Divergent manifestations and outcomes of diffuse crescentic immunoglobulin A nephropathy and pauci-immune crescentic glomerulonephritis on long-term.
Prasad N, Khurana M, Kushwaha R, Patel M, Bhaduaria D, Kaul A, Behera M, Yaccha M, Jain M, Agrawal V
Int Urol Nephrol 2023 Jul;55(7):1847-1855. Epub 2023 Feb 14 doi: 10.1007/s11255-023-03465-9. PMID: 36781681
ANCA-Negative Pauci-immune Necrotizing Glomerulonephritis: A Case Series and a New Clinical Classification.
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
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 glomerulonephritis: does negativity of anti-neutrophilic cytoplasmic antibodies matters?
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

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
Crescentic Glomerulonephritis: an update on Pauci-immune and Anti-GBM diseases.
Kambham N
Adv Anat Pathol 2012 Mar;19(2):111-24. doi: 10.1097/PAP.0b013e318248b7a1. PMID: 22313839
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

Therapy

ANCA-Negative Pauci-immune Necrotizing Glomerulonephritis: A Case Series and a New Clinical Classification.
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
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
Pauci-immune glomerulonephritis: does negativity of anti-neutrophilic cytoplasmic antibodies matters?
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
Pauci-immune crescentic glomerulonephritis in limited cutaneous systemic sclerosis.
Chan PT, Mok CC
Clin Rheumatol 2012 Aug;31(8):1273-7. Epub 2012 May 30 doi: 10.1007/s10067-012-2010-8. PMID: 22644090
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

Prognosis

A potential pathogenic role of interleukin-6 in a child with ANCA-negative pauci-immune crescentic glomerulonephritis: case report and literature review.
Hou L, Yin L, Wu Y, Zhao C, Du Y
BMC Nephrol 2021 Aug 23;22(1):285. doi: 10.1186/s12882-021-02504-w. PMID: 34425760Free PMC Article
Pauci-immune glomerulonephritis: does negativity of anti-neutrophilic cytoplasmic antibodies matters?
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
Clinical course and outcome predictors in pauci-immune ANCA-positive renal-limited vasculitis.
Rupcić V, Jakić M, Rupcić V, Vizjak V, Fijacko M
Acta Clin Croat 2011 Dec;50(4):475-83. PMID: 22649876
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

ANCA-Negative Pauci-immune Necrotizing Glomerulonephritis: A Case Series and a New Clinical Classification.
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
Pauci-immune glomerulonephritis: does negativity of anti-neutrophilic cytoplasmic antibodies matters?
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
Clinical course and outcome predictors in pauci-immune ANCA-positive renal-limited vasculitis.
Rupcić V, Jakić M, Rupcić V, Vizjak V, Fijacko M
Acta Clin Croat 2011 Dec;50(4):475-83. PMID: 22649876
New advances in the pathogenesis of ANCA-associated vasculitides.
Chen M, Kallenberg CG
Clin Exp Rheumatol 2009 Jan-Feb;27(1 Suppl 52):S108-14. PMID: 19646356
ANCA-negative pauci-immune renal vasculitis: histology and outcome.
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

A Meta-Analysis and Cohort Study of Histopathologic and Clinical Outcomes in ANCA-Negative versus -Positive Vasculitis.
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
Pauci-immune crescentic glomerulonephritis in limited cutaneous systemic sclerosis.
Chan PT, Mok CC
Clin Rheumatol 2012 Aug;31(8):1273-7. Epub 2012 May 30 doi: 10.1007/s10067-012-2010-8. PMID: 22644090

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