Clinical and Experimental Nephrology

, Volume 23, Issue 12, pp 1373–1381 | Cite as

Clinicopathological analysis of ANCA-associated glomerulonephritis focusing on plasma cell infiltrate

  • Naoko MasuzawaEmail author
  • Ayako Nishimura
  • Yu Mihara
  • Keiichi Tamagaki
  • Eiichi Konishi
Original article



When we encounter glomerulonephritis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides demonstrating many plasma cell infiltrations, histological overlapping of immunoglobulin G4-related disease (IgG4-RD) often comes into the differential diagnosis. No previous study has focused on the degree of plasma cells in the kidney infiltrate in ANCA-associated glomerulonephritis (ANCA-GN), and the significance of massive plasma cell infiltrate has not been investigated.


To clarify the plasma cell ratio in renal biopsy specimens of ANCA-GN and the histological characteristic of “plasma cell-rich” ANCA-GN, 20 cases of ANCA-GN were reviewed and clinicopathologically analyzed.


Plasma cell ratio was widely distributed between 1.4 and 81%, and the median ratio was 10%. Three patients were categorized in “plasma cell-rich” ANCA-GN, defined as over 45% plasma cell ratio. They tended to include many active glomerular lesions compared to chronic lesions and to display severe tubulointerstitial inflammation. It is suggested that plasma cell-rich ANCA-GN may be acute onset of the disease, and the target of early inflammation may also be in the tubulointerstitial region. Two of the three plasma cell-rich ANCA-GN cases demonstrated numerous IgG4+ cells, but no bird’s-eye pattern fibrosis or obliterative phlebitis.


Plasma cell-rich ANCA-GN is not rare and demonstrates distinct clinicopathological characteristics. This study also reminds us that the presence of the significant number of plasma cells in ANCA-GN, as such, is not a histological diagnostic basis for overlap ANCA-GN and IgG4-related disease.


ANCA Nephritis Plasma cell IgG4 


Compliance with ethical standards

Conflict of interest

The authors have declared that no conflict of interest exists.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee at which the studies were conducted (Ethics committee approval number in Otsu City Hospital 365 and Kyoto Prefectural University of Medicine ERB-C-1285) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.


  1. 1.
    Brodsky S, Nadasdy T. Acute and chronic tubulointerstitial nephritis. In: Jennete JC, Olson JL, Silva FG, D’Agati VD, editors. Heptinstall’s pathology of the kidney. 7th ed. Philadelphia: Wolter Kulwer; 2015. p. 1111–1165.Google Scholar
  2. 2.
    Desvaux D, Gouvello SL, Pastural M, Abtahi M, et al. Acute renal allograft rejections with major interstitial oedema and plasma cell-rich infiltrates: high γ-interferon expression and poor clinical outcome. Nephrol Dial Transplant. 2004;19:933–9.CrossRefGoogle Scholar
  3. 3.
    Yanagita E, Ito T. Evaluation of IgG4/IgG ratio in IgG4-related sclerosing disease using “masking double immunostaining”. Pathol Clin Med. 2010;28:987–92 (in Japanese).Google Scholar
  4. 4.
    Berden AE, Ferrario F, Hagen EC, Jayne DR, et al. Histopathologic classification of ANCA-associated glomerulonephritis. J Am Soc Nephrol. 2010;21:1628–36.CrossRefGoogle Scholar
  5. 5.
    Tipping PG, Kitching AR. Glomerulonephritis, Th1 and Th2: What's new? Clin Exp Immunol. 2005;142:207–15.CrossRefGoogle Scholar
  6. 6.
    Lamprecht P, Kerstein A, Klapa S, Schinke A, et al. Pathogenetic and clinical aspects of anti-neutrophil cytoplasmic autoantibody-associated vasculitis. Front Immunol. 2018. Scholar
  7. 7.
    Jarrot P-A, Kaplanski G. Pathogenesis of ANCA-associated vasculitis: an update. Autoimmune Rev. 2016;15:704–13.CrossRefGoogle Scholar
  8. 8.
    Xiao H, Hu P, Falk RJ, Jennette JC. Overview of the pathogenesis of ANCA-associated vasculitis. Kidney Dis. 2015;1:205–15.CrossRefGoogle Scholar
  9. 9.
    Deshpande V, Zen Y, Chan JKC, Yi EE, et al. Consensus statement of the pathology of IgG4-related disease. Mod Pathol. 2012;25:1181–92.CrossRefGoogle Scholar
  10. 10.
    Della-Torre E, Lansillotta M, Campochiaro C, Bozzalla E, et al. Antineutrophil cytoplasmic antibody positivity in IgG4-related disease: a case report and review of the literature. Medicine. 2016;95(34):e4633. Scholar
  11. 11.
    Su T, Yang L, Cui Z, Wang SX, et al. Concurrent IgG4-related tubulointerstitial nephritis and IgG4 myeloperoxidase-anti-neutrophilic cytoplasmic antibody positive crescentic glomerulonephritis: a case report. Medicine. 2017. Scholar
  12. 12.
    Danlos FX, Rossi GM, Blockmans D, Emmi G, et al. Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: a new overlap syndrome. Autoimmun Rev. 2017;16:1036–43.CrossRefGoogle Scholar
  13. 13.
    Chang SY, Keogh KA, Lewis JE, Ryu JH, et al. IgG4-positive plasma cells in granulomatosis with polyangiitis (Wegener’s): a clinicopathologic and immunohistochemical study on 43 granulomatosis with polyangiitis and 20 control cases. Hum Pathol. 2013;44:2432–7.CrossRefGoogle Scholar
  14. 14.
    Raissian Y, Nasr SH, Larsen CP, Colvin RB, et al. Diagnosis of IgG4-related tubulointerstitial nephritis. J Am Soc Nephrol. 2011;22:1343–52.CrossRefGoogle Scholar
  15. 15.
    Houghton DC, Troxell ML. An abundance of IgG4+ plasma cells is not specific for IgG4-related tubulointerstitial nephritis. Mod Pathol. 2011;24:1480–7.CrossRefGoogle Scholar
  16. 16.
    Yamaguchi Y, Kanetsuna Y, Honda K, Yamanaka N, et al. Characteristic tubulointerstitial nephritis in IgG4-related disease. Hum Pathol. 2012;43:536–49.CrossRefGoogle Scholar

Copyright information

© Japanese Society of Nephrology 2019

Authors and Affiliations

  • Naoko Masuzawa
    • 1
    • 2
    Email author
  • Ayako Nishimura
    • 1
  • Yu Mihara
    • 3
  • Keiichi Tamagaki
    • 3
  • Eiichi Konishi
    • 1
  1. 1.Department of Pathology, Graduate School of Medical ScienceKyoto Prefectural University of MedicineKyotoJapan
  2. 2.Department of Diagnostic PathologyOtsu City HospitalOtsuJapan
  3. 3.Department of Nephrology, Graduate School of Medical ScienceKyoto Prefectural University of MedicineKyotoJapan

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