Skip to main content
SpringerLink
Log in

Proliferative glomerulonephritis with unusual microlamellar organized deposits related to monoclonal immunoglobulin G3 (IgG3) kappa

  • Case Report
  • Published:
CEN Case Reports Aims and scope Submit manuscript

Abstract

A 71-year-old woman presented with massive proteinuria and microhematuria. Renal biopsy showed diffuse global membranoproliferative and endocapillary proliferative lesions with leukocytic infiltration and an irregular duplication of the glomerular basement membrane on light microscopy. Immunofluorescence study showed granular deposits of monoclonal immunoglobulin G3 (IgG3) kappa, C3, and C1q in the glomeruli. Electron microscopy revealed unique structurally organized microlamellar electron-dense deposits. There was no evidence of systemic diseases such as paraproteinemia, cryoglobulinemia, or systemic lupus erythematosus. Following renal biopsy, the oral administration of mizoribine in addition to predonisolone gradually improved the patient’s clinical status. So far, partial remission has continued for a year, and she has not been affected with hematopoietic or lymphoproliferative disorders. We report a case of proliferative glomerulonephritis with unusual microlamellar organized deposits related to monoclonal IgG3 kappa. Our case was immunologically identical to proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID). Therefore, we concluded that our case should be categorized as an atypical form of PGNMID, though it was difficult to diagnose using the usual diagnostic approach to glomerular diseases with organized deposits.

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
Fig. 3

Similar content being viewed by others

References

  1. Iskandar SS, Falk RJ, Jennette JC. Clinical and pathologic features of fibrillary glomerulonephritis. Kidney Int. 1992;42(6):1401–7.

    Article  CAS  PubMed  Google Scholar 

  2. Joh K. Pathology of glomerular deposition diseases. Pathol Int. 2007;57(9):551–65.

    Article  PubMed  Google Scholar 

  3. Herrera GA, Turbat-Herrera EA. Renal diseases with organized deposits: an algorithmic approach to classification and clinicopathologic diagnosis. Arch Pathol Lab Med. 2010;134(4):512–31.

    PubMed  Google Scholar 

  4. Muchtar E, Magen H, Gertz MA. How I treat cryoglobulinemia. Blood. 2017;129(3):289–98.

    Article  CAS  PubMed  Google Scholar 

  5. Bridoux F, Hugue V, Coldefy O, Goujon JM, Bauwens M, Sechet A, Preud’Homme JL, Touchard G. Fibrillary glomerulonephritis and immunotactoid (microtubular) glomerulopathy are associated with distinct immunologic features. Kidney Int. 2002;62(5):1764–75.

    Article  CAS  PubMed  Google Scholar 

  6. Motwani SS, Herlitz L, Monga D, Jhaveri KD, Lam AQ, American Society of Nephrology Onco-Nephrology F. Paraprotein-related kidney disease: glomerular diseases associated with paraproteinemias. Clin J Am Soc Nephrol. 2016;11(12):2260–72.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Nasr SH, Valeri AM, Cornell LD, Fidler ME, Sethi S, Leung N, Fervenza FC. Fibrillary glomerulonephritis: a report of 66 cases from a single institution. Clin J Am Soc Nephrol. 2011;6(4):775–84.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Nasr SH, Markowitz GS, Stokes MB, Seshan SV, Valderrama E, Appel GB, Aucouturier P, D’Agati VD. Proliferative glomerulonephritis with monoclonal IgG deposits: a distinct entity mimicking immune-complex glomerulonephritis. Kidney Int. 2004;65(1):85–96.

    Article  CAS  PubMed  Google Scholar 

  9. Nasr SH, Satoskar A, Markowitz GS, Valeri AM, Appel GB, Stokes MB, Nadasdy T, D’Agati VD. Proliferative glomerulonephritis with monoclonal IgG deposits. J Am Soc Nephrol. 2009;20(9):2055–64.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Doshi M, Lahoti A, Danesh FR, Batuman V, Sanders PW, American Society of Nephrology Onco-Nephrology F. Paraprotein-related kidney disease: kidney injury from paraproteins—what determines the site of injury? Clin J Am Soc Nephrol. 2016;11(12):2288–94.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Bridoux F, Leung N, Hutchison CA, Touchard G, Sethi S, Fermand JP, Picken MM, Herrera GA, Kastritis E, Merlini G, Roussel M, Fervenza FC, Dispenzieri A, Kyle RA, Nasr SH, International K, Monoclonal Gammopathy Research G. Diagnosis of monoclonal gammopathy of renal significance. Kidney Int. 2015;87(4):698–711.

    Article  CAS  PubMed  Google Scholar 

  12. Joh K, Aizawa S, Takahashi T, Hatakeyama M, Muto S, Asano Y, Shimizu H, Suzuki R. Microlamellar structures in lobular glomerulonephritis associated with monoclonal IgG lambda paraproteinemia. A case report and review of the literature. Acta Pathol Jpn. 1990;40(12):913–21.

    CAS  PubMed  Google Scholar 

Download references

Acknowledgements

We thank Dr. Masakazu Akashi (Department of Nephrology, Kita-Asaka station clinic, Saitama, Japan) for helpful comments and Ms. Arimi Ishikawa (Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan) for expert technical assistance.

Author information

Author notes

  1. Daisuke Takada

    Present address: Department of Nephrology, Tama Nagayama Takada Clinic, Tokyo, Japan

Authors and Affiliations

  1. Department of Nephrology, Nippon Medical School, 1-1-5, Sendagi, Bunkyo-ku, Tokyo, 113-8602, Japan

    Akiko Mii & Shuichi Tsuruoka

  2. Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan

    Akira Shimizu

  3. Department of Nephrology, Kita-Asaka Station Clinic, Saitama, Japan

    Daisuke Takada

Authors
  1. Akiko Mii
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Akira Shimizu
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Daisuke Takada
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Shuichi Tsuruoka
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Akiko Mii.

Ethics declarations

Conflict of interest

The authors declare that they have no competing interests.

Human and animal rights

All procedures performed in this case report were in accordance with the ethical standards of the institutional committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. This study does not contain any studies with animals performed by any of the authors.

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Mii, A., Shimizu, A., Takada, D. et al. Proliferative glomerulonephritis with unusual microlamellar organized deposits related to monoclonal immunoglobulin G3 (IgG3) kappa. CEN Case Rep 7, 320–324 (2018). https://doi.org/10.1007/s13730-018-0351-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13730-018-0351-0

Keywords

Search

Navigation