A 74-year-old Japanese woman was referred to our department because of anasarca and massive proteinuria. She was clinically diagnosed with nephrotic syndrome, and renal biopsy showed membranoproliferative glomerulonephritis accompanied by marked glomerular infiltration with macrophages and full-house immunofluorescence glomerular deposition. Furthermore, randomly arranged nonbranching fibrils, approximately 12 nm in diameter, were found by electron microscopy, and immunostaining for DnaJ homolog subfamily B member 9 (DNAJB9), a recently identified diagnostic biomarker of fibrillary glomerulonephritis (FGN), showed positive result, thereby confirming the diagnosis of FGN. Steroid treatment was initiated, and she obtained complete remission of nephrotic syndrome and has maintained it. FGN is an uncommon form of glomerular disease, and reported cases of DNAJB9-positive FGN among Asians, particularly among Japanese population, are rare. There have been no established therapeutic regimens and its renal prognosis is generally unfavorable. The present case suggests that some patients with FGN can achieve favorable clinical outcomes through steroid monotherapy.
This is a preview of subscription content, access via your institution.
Buy single article
Instant access to the full article PDF.
Tax calculation will be finalised during checkout.
Subscribe to journal
Immediate online access to all issues from 2019. Subscription will auto renew annually.
Tax calculation will be finalised during checkout.
Nasr SH, Fogo AB. New developments in the diagnosis of fibrillary glomerulonephritis. Kidney Int. 2019;96(3):581–92. https://doi.org/10.1016/j.kint.2019.03.021.
Alpers CE, Kowalewska J. Fibrillary glomerulonephritis and immunotactoid glomerulopathy. J Am Soc Nephrol. 2008;19(1):34–7. https://doi.org/10.1681/ASN.2007070757.
Nasr SH, Vrana JA, Dasari S, Bridoux F, Fidler ME, Kaaki S, et al. DNAJB9 is a specific immunohistochemical marker for fibrillary glomerulonephritis. Kidney Int Rep. 2018;3(1):56–64. https://doi.org/10.1016/j.ekir.2017.07.017.
Dasari S, Alexander MP, Vrana JA, Theis JD, Mills JR, Negron V, et al. DnaJ heat shock protein family B member 9 is a novel biomarker for fibrillary GN. J Am Soc Nephrol. 2018;29(1):51–6. https://doi.org/10.1681/ASN.2017030306.
Andeen NK, Yang HY, Dai DF, MacCoss MJ, Smith KD. DnaJ homolog subfamily B member 9 is a putative autoantigen in fibrillary GN. J Am Soc Nephrol. 2018;29(1):231–9. https://doi.org/10.1681/ASN.2017050566.
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. https://doi.org/10.1155/2020/6343521.
Whitaker G, Brown EA, Moss J, Woodrow D, Frankel A. Fibrillary glomerulonephritis occurring in association with hereditary angioneurotic oedema, pernicious anaemia and hypothyroidism. Nephrol Dial Transplant. 1998;13(7):1822–4. https://doi.org/10.1093/ndt/13.7.1822.
Rosenstock JL, Markowitz GS. Fibrillary glomerulonephritis: an update. Kidney Int Rep. 2019;4(7):917–22. https://doi.org/10.1016/j.ekir.2019.04.013.
Nasr SH, Valeri AM, Cornell LD, Fidler ME, Sethi S, Leung N, et al. Fibrillary glomerulonephritis: a report of 66 cases from a single institution. Clin J Am Soc Nephrol. 2011;6(4):775–84. https://doi.org/10.2215/CJN.08300910.
Sekulic M, Nasr SH, Grande JP, Cornell LD. Histologic regression of fibrillary glomerulonephritis: the first report of biopsy-proven spontaneous resolution of disease. Clin Kidney J. 2017;10(6):738–41. https://doi.org/10.1093/ckj/sfx045.
Andeen NK, Troxell ML, Riazy M, Avasare RS, Lapasia J, Jefferson JA, et al. Fibrillary glomerulonephritis: clinicopathologic features and atypical cases from a multi-institutional cohort. Clin J Am Soc Nephrol. 2019;14(12):1741–50. https://doi.org/10.2215/CJN.03870319.
Payan Schober F, Jobson MA, Poulton CJ, Singh HK, Nickeleit V, Falk RJ, et al. Clinical features and outcomes of a racially diverse population with fibrillary glomerulonephritis. Am J Nephrol. 2017;45(3):248–56. https://doi.org/10.1159/000455390.
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(4):1450–61. https://doi.org/10.1046/j.1523-1755.2003.00853.x.
Kalbermatter SA, Marone C, Casartelli D, Hausberg M, Banfi G, Mihatsch M, et al. Outcome of fibrillary glomerulonephritis. Swiss Med Wkly. 2012;142:w13578. https://doi.org/10.4414/smw.2012.13578.
Javaugue V, Karras A, Glowacki F, McGregor B, Lacombe C, Goujon JM, et al. Long-term kidney disease outcomes in fibrillary glomerulonephritis: a case series of 27 patients. Am J Kidney Dis. 2013;62(4):679–90. https://doi.org/10.1053/j.ajkd.2013.03.031.
Dickenmann M, Schaub S, Nickeleit V, Mihatsch M, Steiger J, Brunner F. Fibrillary glomerulonephritis: early diagnosis associated with steroid responsiveness. Am J Kidney Dis. 2002;40(3):E9. https://doi.org/10.1053/ajkd.2002.34933.
We thank Ms. Yoshie Wakatsuki and Professor Hiroshi Hirano, at the Department of Pathology of Tokyo Medical University Hachioji Medical Center, for expert technical assistance and advice regarding the pathological analyses
Conflict of interest
All the authors have declared no competing interest.
This article does not contain any studies with human participants or animals performed by any of the authors.
Informed consent was obtained from the patient for publication.
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
About this article
Cite this article
Uchida, T., Komatsu, S., Sakai, T. et al. Complete remission of DnaJ homolog subfamily B member 9-positive fibrillary glomerulonephritis following steroid monotherapy in an elderly Japanese woman. CEN Case Rep (2021). https://doi.org/10.1007/s13730-021-00585-y
- DnaJ homolog subfamily B member 9 (DNAJB9)
- Fibrillary glomerulonephritis (FGN)
- Nephrotic syndrome