Skip to main content

Advertisement

SpringerLink
Log in

A historical study of American patients with anti-neutrophil cytoplasmic antibody negative pauci-immune glomerulonephritis

  • Original Article
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Anti-neutrophil cytoplasmic antibodies (ANCA) play an important role in the pathogenesis of ANCA-associated vasculitis. The lack of ANCA antibodies may indicate a variation in clinical presentation and outcomes of this disease. We identified 74 adult patients between 1995 and 2009 with the diagnosis of pauci-immune glomerulonephritis. Demographics, histological features, and treatment outcomes were compared between ANCA-positive and ANCA-negative patients. These factors were correlated with renal function at presentation and follow-up. Of the 74 patients, 57 were ANCA-positive, and 17 were ANCA-negative. Demographics and mean Birmingham Vasculitis Activity Score were similar between ANCA-negative and ANCA-positive patients at presentation. Renal function was significantly worse at presentation in the ANCA-negative patients (eGFR 16.59 vs. 31.89 ml/min/1.73 m2, p = 0.03). Patients in the ANCA-negative group had a significantly higher interstitial fibrosis score compared to the ANCA-positive group (2.1 vs.1.6, p = 0.04). The median time to remission was shorter in the ANCA-negative patients (51 vs. 78 days, p = 0.01). Long-term renal function and 1-year patient and renal survival were similar between ANCA-negative and ANCA-positive patients. Baseline eGFR, percentage of normal glomeruli, glomerular sclerosis, and tubulointerstitial scarring predicted eGFR at 1 year in both groups similarly. This is the first historical review of American patients with pauci-immune glomerulonephritis, comparing patients with ANCA-negative and ANCA-positive serology. Although ANCA-negative patients present with lower eGFR and more interstitial fibrosis, 1-year and long-term outcomes in both groups are similar.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Falk RJ, Jennette JC (1988) Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis. N Engl J Med 318(25):1651–1657

    Article  CAS  PubMed  Google Scholar 

  2. Booth AD, Almond MK, Burns A et al (2003) Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis 41(4):776–784

    Article  PubMed  Google Scholar 

  3. Jennette JC, Falk RJ (1997) Small-vessel vasculitis. N Engl J Med 337(21):1512–1523

    Article  CAS  PubMed  Google Scholar 

  4. Jennette JC, Falk RJ, Gasim AH (2011) Pathogenesis of antineutrophil cytoplasmic autoantibody vasculitis. Curr Opin Nephrol Hypertens 20(3):263–270

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Hedger N, Stevens J, Drey N, Walker S, Roderick P (2000) Incidence and outcome of pauci-immune rapidly progressive glomerulonephritis in wessex, UK: a 10-year retrospective study. Nephrol Dial Transplant 15(10):1593–1599

    Article  CAS  PubMed  Google Scholar 

  6. Chen M, Yu F, Wang SX, Zou WZ, Zhao MH, Wang HY (2007) Antineutrophil cytoplasmic autoantibody-negative pauci-immune crescentic glomerulonephritis. J Am Soc Nephrol 18(2):599–605

    Article  PubMed  Google Scholar 

  7. Hung PH, Chiu YL, Lin WC et al (2006) Poor renal outcome of antineutrophil cytoplasmic antibody negative pauci-immune glomerulonephritis in Taiwanese. J Formos Med Assoc 105(10):804–812

    Article  CAS  PubMed  Google Scholar 

  8. Eisenberger U, Fakhouri F, Vanhille P et al (2005) ANCA-negative pauci-immune renal vasculitis: histology and outcome. Nephrol Dial Transplant 20(7):1392–1399

    Article  PubMed  Google Scholar 

  9. Jennette JC, Falk RJ, Andrassy K et al (1994) Nomenclature of systemic vasculitides. proposal of an international consensus conference. Arthritis Rheum 37(2):187–192

    Article  CAS  PubMed  Google Scholar 

  10. Stone JH, Hoffman GS, Merkel PA et al (2001) A disease-specific activity index for Wegener’s granulomatosis: modification of the birmingham vasculitis activity score. International Network for the Study of the Systemic Vasculitides (INSSYS). Arthritis Rheum 44(4):912–920

    Article  CAS  PubMed  Google Scholar 

  11. Levey AS, Stevens LA, Schmid CH et al (2009) A new equation to estimate glomerular filtration rate. Ann Intern Med 150(9):604–612

    Article  PubMed  PubMed Central  Google Scholar 

  12. Bajema IM, Hagen EC, Hermans J et al (1999) Kidney biopsy as a predictor for renal outcome in ANCA-associated necrotizing glomerulonephritis. Kidney Int 56(5):1751–1758

    Article  CAS  PubMed  Google Scholar 

  13. Hauer HA, Bajema IM, Van Houwelingen HC et al (2002) Determinants of outcome in ANCA-associated glomerulonephritis: a prospective clinico-histopathological analysis of 96 patients. Kidney Int 62(5):1732–1742

    Article  PubMed  Google Scholar 

  14. de Lind van Wijngaarden RA, Hauer HA, Wolterbeek R et al (2006) Clinical and histologic determinants of renal outcome in ANCA-associated vasculitis: a prospective analysis of 100 patients with severe renal involvement. J Am Soc Nephrol 17(8):2264–2274

    Article  PubMed  Google Scholar 

  15. Day CJ, Howie AJ, Nightingale P et al (2010) Prediction of ESRD in pauci-immune necrotizing glomerulonephritis: quantitative histomorphometric assessment and serum creatinine. Am J Kidney Dis 55(2):250–258

    Article  PubMed  PubMed Central  Google Scholar 

  16. Carvalho D, Savage CO, Isenberg D, Pearson JD (1999) IgG anti-endothelial cell autoantibodies from patients with systemic lupus erythematosus or systemic vasculitis stimulate the release of two endothelial cell-derived mediators, which enhance adhesion molecule expression and leukocyte adhesion in an autocrine manner. Arthritis Rheum 42(4):631–640

    Article  CAS  PubMed  Google Scholar 

  17. Guilpain P, Mouthon L (2008) Antiendothelial cells autoantibodies in vasculitis-associated systemic diseases. Clin Rev Allergy Immunol 35(1–2):59–65

    Article  CAS  PubMed  Google Scholar 

  18. Sebastian JK, Mahr AD, Ahmed SS et al (2007) Antiendothelial cell antibodies in patients with Wegener’s granulomatosis: prevalence and correlation with disease activity and manifestations. J Rheumatol 34(5):1027–1031

    CAS  PubMed  Google Scholar 

  19. Gobel U, Eichhorn J, Kettritz R et al (1996) Disease activity and autoantibodies to endothelial cells in patients with Wegener’s granulomatosis. Am J Kidney Dis 28(2):186–194

    Article  CAS  PubMed  Google Scholar 

  20. Kain R, Exner M, Brandes R et al (2008) Molecular mimicry in pauci-immune focal necrotizing glomerulonephritis. Nat Med 14(10):1088–1096

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Roth AJ, Brown MC, Smith RN et al (2012) Anti-LAMP-2 antibodies are not prevalent in patients with antineutrophil cytoplasmic autoantibody glomerulonephritis. J Am Soc Nephrol 23(3):545–555

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Kain R, Tadema H, McKinney EF et al (2012) High prevalence of autoantibodies to hLAMP-2 in anti-neutrophil cytoplasmic antibody-associated vasculitis. J Am Soc Nephrol 23(3):556–566

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Roth AJ, Ooi JD, Hess JJ et al (2013) Epitope specificity determines pathogenicity and detectability in ANCA-associated vasculitis. J Clin Invest 123(4):1773–1783

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Nephrology, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD, USA

    Shivani Shah & Duvuru Geetha

  2. Kidney Specialists of Southern Nevada, 100 N Green Valley pkwy, Henderson, NV, USA

    John Havill

  3. Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD, USA

    M. Hafizur Rahman

  4. Division of Nephrology, Department of Medicine, Johns Hopkins Bayview Medical Center, 301 Mason Lord Drive, Suite 2500, Baltimore, MD, 21224, USA

    Shivani Shah

Authors
  1. Shivani Shah
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. John Havill
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. M. Hafizur Rahman
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Duvuru Geetha
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Shivani Shah.

Ethics declarations

Conflict of interest

Duvuru Geetha, MD has served as a consultant for Genentech.

Shivani Shah, MD, John Havill, MD, and M. Hafizur Rahman, MD have no conflicts of interest to disclose.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Shah, S., Havill, J., Rahman, M.H. et al. A historical study of American patients with anti-neutrophil cytoplasmic antibody negative pauci-immune glomerulonephritis. Clin Rheumatol 35, 953–960 (2016). https://doi.org/10.1007/s10067-015-3086-8

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-015-3086-8

Keywords

Search

Navigation