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Clinicopathological characteristics and outcome of patients with fibrillary glomerulonephritis: DNAJB9 is a valuable histologic marker

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Abstract

Background

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease. DNAJB9 as a new reliable diagnostic marker for the diagnosis of FGN was discovered recently. To investigate the clinicopathological features and prognosis of DNAJB9-positive FGN, we report on a case series in a single center in China.

Methods

DNAJB9 immunohistochemistry was performed on renal biopsy samples from patients with FGN (n = 7), and non-FGN glomerular diseases (n = 27) were used as controls. The patients with DNAJB9-positive FGN were retrospectively analyzed.

Results

Strong DNAJB9 staining of glomerular extracellular deposits was observed in 6 cases of originally diagnosed FGN. One man and 5 women with a median age of 26 years were studied. The patients presented with renal insufficiency in 1 case, proteinuria in 6 cases, nephrotic syndrome in 3 cases, and microscopic hematuria in 2 cases. The histologic pattern was mesangial proliferative glomerulonephritis in 1 case and membranoproliferative glomerulonephritis in 5 cases. The glomerular deposits stained for polytypic IgG and both kappa and lambda in 3 cases, polytypic IgG without kappa or lambda in 1, monotypic IgG1-kappa in 1 and IgG1-lambda in 1. Extraglomerular deposits were identified in all cases. Congo red positivity was observed in 3 cases. All of the patients received renin–angiotensin–aldosterone system blockade and 5 of them received glucocorticoid and/or immunosuppression. At a median time of 36.2 months after biopsy, 2 cases had partial remission, 3 cases displayed no remission, and 1 case progressed to end-stage renal disease.

Conclusions

Extraglomerular deposits in the FGN were common. Monotypic FGN was found in young patients with a favorable renal outcome.

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Funding

This work was supported by National Key Research and Development Program of China (2016YFC0901202), the National Natural Science Foundation of China (81800629).

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Authors and Affiliations

  1. National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, East 305 Zhongshan Road, Nanjing, 210002, China

    Shaoshan Liang, Dacheng Chen, Dandan Liang, Feng Xu, Mingchao Zhang, Fan Yang, Xiaodong Zhu, Ping Li & Caihong Zeng

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  1. Shaoshan Liang
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  2. Dacheng Chen
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  3. Dandan Liang
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  4. Feng Xu
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  5. Mingchao Zhang
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  6. Fan Yang
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  7. Xiaodong Zhu
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  8. Ping Li
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  9. Caihong Zeng
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Contributions

All authors have reviewed and contributed to this manuscript. The individual contribution of each co-author was as follows: SL, CZ contributed to research idea and study design; SL, DC, DL, FX, MZ, FY, XZ, PL contributed to data acquisition; SL, CZ, MZ contributed to pathological analysis; SL, DC, MZ contributed to data analysis and interpretation; CZ contributed to supervision and mentorship.

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Correspondence to Caihong Zeng.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Liang, S., Chen, D., Liang, D. et al. Clinicopathological characteristics and outcome of patients with fibrillary glomerulonephritis: DNAJB9 is a valuable histologic marker. J Nephrol 34, 883–892 (2021). https://doi.org/10.1007/s40620-020-00783-4

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  • DOI: https://doi.org/10.1007/s40620-020-00783-4

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