Skip to main content

Advertisement

SpringerLink
Log in

A case of juvenile-onset fibrillary glomerulonephritis diagnosed by mass spectrometry and immunohistochemistry of DNAJB9

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

Abstract

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease. FGN is characterized by the deposition of randomly arranged, nonbranching microfibrils in the mesangium and glomerular basement membrane. The discovery of DNAJ homolog subfamily B member 9 (DNAJB9) in 2017 was a breakthrough, and DNAJB9 has been proven to be extremely useful for the definitive diagnosis of FGN. While FGN often occurs in middle-aged individuals, this case was diagnosed at a relatively young age of 17. We performed renal biopsy, and light microscopic study revealed mesangial proliferation with expansion and subepithelial deposits. Electron microscopic study showed glomerular deposition of randomly oriented nonbranching fibrils with a mean of 20 nm. However, direct first scarlet stain for amyloidosis was weakly positive. Therefore, we confirmed the diagnosis of FGN and eliminated the presence of amyloidosis with mass spectrometry. This is the first case in Japan in which the complication of amyloidosis was ruled out with mass spectrometry and FGN was diagnosed using immunostaining and mass spectrometry of DNAJB9. We began treatment with cyclosporine A. One and a half years after the start of the treatment, kidney function continues to be normal.

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. Rosenmann E, Eliakim M. Nephrotic syndrome associated with amyloid-like glomerular deposits. Nephron. 1977;18:301–8.

    Article  PubMed  CAS  Google Scholar 

  2. Ivanyi B, Degrell P. Fibrillary glomerulonephritis and immunotactoid glomerulopathy. Nephrol Dial Transplant. 2004;19:2166–70.

    Article  PubMed  Google Scholar 

  3. Rosenstock JL, Markowitz GS, Valeri AM, Sacchi G, Appel GB, D’Agati VD. Fibrillary and immunotactoid glomerulonephritis: distinct entities with different clinical and pathologic features. Kidney Int. 2003;63:1450–61.

    Article  PubMed  Google Scholar 

  4. Nasr SH, Vrana JA, Dasari S, et al. DNAJB9 is a specific immunohistochemical marker for fibrillary glomerulonephritis. Kidney Int Rep. 2017;3:56–64.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Nasr SH, Valeri AM, Cornell LD, et al. Fibrillary glomerulonephritis: a report of 66 cases from a single institution. Clin J Am Soc Nephrol. 2011;6:775–84.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Andeen NK, Troxell ML, Avasare RS, et al. Fibrillary glomerulonephritis. Clinicopathologic features and atypical cases from a multi-institutional cohort. Clin J Am Soc Nephrol. 2019;14:1741–50.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  7. Rosenstock JL, Markowitz GS. Fibrillary glomerulonephritis: an update. Kidney Int Rep. 2019;4:917–22.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Asakawa T, Asou M, Hara S, Ehara T, Araki M. Fibrillary glomerulopathy with a high level of myeloperoxidase-ANCA: a case report. Case Rep Nephrol. 2020;2020:6343521.

    PubMed  PubMed Central  Google Scholar 

  9. Uchida T, Komatsu S, Sakai T, Kojima A. Complete remission of DnaJ homolog subfamily B member 9-positive fibrillary glomerulonephritis following steroid monotherapy in an elderly Japanese woman. CEN Case Rep. 2020;10:442–7.

    Article  Google Scholar 

  10. Alexander MP, Dasari S, Vrana JA, et al. Congophilic fibrillary glomerulonephritis: a case series. Am J Kidney Dis. 2018;72:325–36.

    Article  PubMed  Google Scholar 

  11. Said SM, Rocha AB, Royal V, et al. Immunoglobulin-negative DNAJB9-associated fibrillary glomerulonephritis: a report of 9 cases. Am J Kidney Dis. 2021;77:454–8.

    Article  PubMed  CAS  Google Scholar 

  12. Menon S, Zeng X, Valentini R. Fibrillary glomerulonephritis and renal failure in a child with systemic lupus erythematosus. Pediatr Nephrol. 2009;24:1577–81.

    Article  PubMed  Google Scholar 

  13. Bahrami D, Henegar JR, Baliga R. Fibrillary glomerulopathy in a 10-year-old female. Pediatr Nephrol. 2001;16:916–8.

    Article  PubMed  CAS  Google Scholar 

  14. Bircan Z, Toprak D, Kilicaslan I, et al. Factor H deficiency and fibrillary glomerulopathy. Nephrol Dial Transplant. 2004;19:727–30.

    Article  PubMed  CAS  Google Scholar 

  15. Chung WY, Lee SY, Joo JE. Fibrillary glomerulonephritis in a 9-year-old girl. Nephron. 1995;69:79–82.

    Article  PubMed  CAS  Google Scholar 

  16. Mazzucco G, Casanova S, Donini U, et al. Glomerulonephritis with organized deposits: a new clinicopathological entity? Light-, electron-microscopic and immunofluorescence study of 12 cases. Am J Nephrol. 1990;10:21–30.

    Article  PubMed  CAS  Google Scholar 

  17. Shim YH, Lee SJ, Sung SH. A case of fibrillary glomerulonephritis with unusual IgM deposits and hypocomplementemia. Pediatr Nephrol. 2008;23:1163–6.

    Article  PubMed  Google Scholar 

  18. Takemura T, Yoshioka K, Akano N, et al. Immunotactoid glomerulopathy in a child with Down syndrome. Pediatr Nephrol. 1993;7:86–8.

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgements

We would like to thank Mr. Norihiko Suzuki, Ms. Naoko Asano, Ms. Yuriko Sawa, and Ms. Ryoko Sakamoto (Nagoya University) for excellent technical assistance with light microscopy, immunofluorescence, and electron microscopy. Also, we thank Ms. Naomi Kuwahara and Ms. Arimi Ishikawa (Nippon Medical School) for DNAJB9 immunohistochemistry and mass spectrometry.

Author information

Authors and Affiliations

  1. Department of Nephrology, Anjo Kosei Hospital, 28, higashihirokute, anjocho, Anjo, Aichi, 446-8602, Japan

    Keita Hattori, Ryo Shimizu, Shoichiro Tanaka, Takashi Terashima, Miya Hiramatsu, Taishi Shimomura, Takeshi Ito, Takatoshi Morinaga & Hirofumi Tamai

  2. Department of Analytic Human Pathology, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo, 113-8603, Japan

    Mika Terasaki & Akira Shimizu

  3. Department of Nephrology, Masuko Memorial Hospital, 35-28 Takegashicho, Nakamura-ku, Nagoya, Aichi, 453-0016, Japan

    Kunio Morozumi

Authors
  1. Keita Hattori
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ryo Shimizu
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Shoichiro Tanaka
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Takashi Terashima
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Miya Hiramatsu
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Taishi Shimomura
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Takeshi Ito
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Takatoshi Morinaga
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Mika Terasaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Akira Shimizu
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Kunio Morozumi
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Hirofumi Tamai
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Keita Hattori.

Ethics declarations

Conflict of interest

All the authors have declared no competing interests.

Ethics approval

This paper does not contain any studies with human participants or animals performed by any of the authors.

Informed consent

Informed consent was obtained from the patient in the case report.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hattori, K., Shimizu, R., Tanaka, S. et al. A case of juvenile-onset fibrillary glomerulonephritis diagnosed by mass spectrometry and immunohistochemistry of DNAJB9. CEN Case Rep 11, 412–416 (2022). https://doi.org/10.1007/s13730-022-00693-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13730-022-00693-3

Keywords

Search

Navigation