纤维性肾小球肾炎的发病机制及诊治研究进展

doi:10.12372/jcp.2026.25e0295

Abstract

Abstract:

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease with unknown etiology. Member of DNAJ heat shock protein family B9 (DNAJB9) protein may be involved in its pathogenesis. The clinical manifestations of FGN are mostly nephrotic syndrome, which can be combined with hematuria, hypertension and renal insufficiency. A few patients show rapid progressive glomerulonephritis. It has important diagnostic value by electron microscopic examination of renal tissue combined with immunohistochemistry of DNAJB9 in FGN patients.Unfortunately, there is no specific therapy at present, and the prognosis is poor. In the future, it is urgent to explore the potential therapeutic targets of FGN to improve the quality of life of FGN patients. This article reviews the possible pathogenesis and clinical characteristics of FGN.

Key words: fibrillary glomerulonephritis, member of DNAJ heat shock protein family B9, renal biopsy, electron microscope, nephrotic syndrome

CLC Number:

ZHANG Yuanyuan, ZHAO Dean. Advances in pathogenesis, diagnosis and treatment of fibrillary glomerulonephritis[J].Journal of Clinical Pediatrics, 2026, 44(2): 167-174.

References 50

[1]	Rosenmann E, Eliakim M. Nephrotic syndrome associated with amyloid-like glomerular deposits[J]. Nephron, 1977, 18(5): 301-308. doi: 10.1159/000180846 pmid: 865657
[2]	Duffy JL, Khurana E, Susin M, et al. Fibrillary renal deposits and nephritis[J]. Am J Pathol, 1983, 113(3): 279-290. pmid: 6359891
[3]	Alpers CE, Rennke HG, Hopper J Jr, et al. Fibrillary glomerulonephritis: an entity with unusual immuno-fluorescence features[J]. Kidney Int, 1987, 31(3): 781-789. pmid: 3106698
[4]	Liang S, Chen D, Liang D, et al. Clinicopathological characteristics and outcome of patients with fibrillary glomerulonephritis: DNAJB9 is a valuable histologic marker[J]. J Nephrol, 2021, 34(3): 883-892. doi: 10.1007/s40620-020-00783-4
[5]	Nasr SH, Fogo AB. New developments in the diagnosis of fibrillary glomerulonephritis[J]. Kidney Int, 2019, 96(3): 581-592. doi: S0085-2538(19)30407-7 pmid: 31227146
[6]	Klomjit N, Alexander MP, Zand L. Fibrillary glomeru-lonephritis and DnaJ homolog subfamily B member 9 (DNAJB9)[J]. Kidney360, 2020, 1(9): 1002-1013.
[7]	徐秋郁, 文煜冰, 崔皓园, 等. 临床进展相对缓慢的纤维性肾小球肾炎1例[J]. 中华内科杂志, 2024, 63(4): 412-415.
	Xu QY, Wen YB, Cui HY, et al. A case of fibrillary glomerulonephritis with relatively slow clinical progression[J]. Zhonghua Neike Zazhi, 2024, 63(4): 412-415.
[8]	Saleem M, Khan MM, Iftikhar H. Fibrillary glomeru-lonephritis leading to end-stage renal disease in the absence of active or chronic hepatitis C infection: current insights[J]. Cureus, 2024, 16(6): e61831.
[9]	Sethi S, Theis JD, Vrana JA, et al. Laser microdissection and proteomic analysis of amyloidosis, cryoglobulinemic GN, fibrillary GN, and immunotactoid glomerulopathy[J]. Clin J Am Soc Nephro, 2013, 8 (6): 915-21. doi: 10.2215/CJN.07030712 pmid: 23411427
[10]	Nasr SH, Vrana JA, Dasari S, et al. DNAJB9 is a specific immunohistochemical marker for fibrillary glomerulonephritis[J]. Kidney Int Rep, 2017, 3(1): 56-64.
[11]	Dasari S, Alexander MP, Vrana JA, et al. DnaJ heat shock protein family B member 9 is a novel biomarker for fibrillary GN[J]. J Am Soc Nephrol, 2018, 29(1): 51-56. doi: 10.1681/ASN.2017030306 pmid: 29097623
[12]	Gambella A, Pitino C, Barreca A, et al. DNAJB9 is a reliable immunohistochemical marker of fibrillary glomerulonephritis: evaluation of diagnostic efficacy in a large series of kidney biopsies[J]. Biomedicines, 2022, 10(9): 2102. doi: 10.3390/biomedicines10092102
[13]	Nasr SH, Sirac C, Bridoux F, et al. Heavy chain fibrillary glomerulonephritis: a case report[J]. Am J Kidney Dis, 2019, 74(2): 276-280. doi: S0272-6386(19)30162-3 pmid: 30955945
[14]	El Ters M, Bobart SA, Cornell LD, et al. Recurrence of DNAJB9-Positive fibrillary glomerulonephritis after kidney transplantation: a case series[J]. Am J Kidney Dis, 2020, 76(4): 500-510. doi: S0272-6386(20)30571-0 pmid: 32414663
[15]	杨雪. DNAJ热休克蛋白家族成员B9的研究进展[J]. 肾脏病与透析肾移植杂志, 2022, 31(4): 368-372.
	Yang X. Research progress on DNAJ heat shock protein family member B9[J]. Shenzangbing Yu Touxi Shenyizhi Zazhi, 2022, 31(4): 368-372.
[16]	Merquiol E, Uzi D, Mueller T, et al. HCV causes chronic endoplasmic reticulum stress leading to adaptation and interference with the unfolded protein response[J]. PLoS One, 2011, 6(9): e24660. doi: 10.1371/journal.pone.0024660
[17]	Behnke J, Mann MJ, Scruggs FL, et al. Members of the HSP70 family recognize distinct types of sequences to execute ER quality control[J]. Mol Cell, 2016, 63(5): 739-752. doi: 10.1016/j.molcel.2016.07.012 pmid: 27546788
[18]	Avasare RS, Robinson BA, Nelson J, et al. DNAJB9 is not transcriptionally upregulated in the glomerulus in fibrillary glomerulonephritis[J]. Kidney Int Rep, 2019, 5(3): 368-372.
[19]	Andeen NK, Smith KD, Vasilescu ER, et al. Fibrillary glomerulonephritis is associated with HLA-DR7 and HLA-B35 antigens[J]. Kidney Int Rep, 2020, 5(8): 1325-1327. doi: 10.1016/j.ekir.2020.05.010 pmid: 32775835
[20]	El Ters M, Gandhi MJ, Moyer AM, et al. Letter regarding "Fibrillary glomerulonephritis is associated with HLA-DR7 and HLA-B35 antigens"[J]. Kidney Int Rep, 2020, 5 (10): 1840-1841.
[21]	Andeen NK, Smith KD, Vasilescu ER, et al. Fibrillary glomerulonephritis is associated with HLA-DR7 and HLA-B35 antigens[J]. Kidney Int Rep, 2020, 5 (8): 1325-1327. doi: 10.1016/j.ekir.2020.05.010 pmid: 32775835
[22]	Watanabe K, Nakai K, Hosokawa N, et al. A case of fibrillary glomerulonephritis with fibril deposition in the arteriolar wall and a family history of renal disease[J]. Case Rep Nephrol Dial, 2017, (1): 26-33. doi: 10.1159/000468517 pmid: 28503551
[23]	Ying T, Hill P, Desmond M, et al. Fibrillary glome-rulonephritis: An apparent familial form?[J]. Nephrology, 2015, 20 (7): 506-509. doi: 10.1111/nep.2015.20.issue-7
[24]	Jeyabalan A, Batal I, Piras D, et al. Familial fibrillary glomerulonephritis in living related kidney transplantation[J]. Kidney Int Rep, 2020, 6(1): 239-242.
[25]	王刘伟, 于露, 翟子涵, 等. 表现为新月体性肾小球肾炎的纤维性肾小球病[J]. 肾脏病与透析肾移植杂志, 2024, 33(1): 92-96.
	Wang LW, Yu L, Zhai ZH, et al. Fibrous glomerulopathy presenting as crescentic glomerulonephritis[J]. Shenzangbing Yu Touxi Shenyizhi Zazhi, 2024, 33(1): 92-96.
[26]	Alexander MP, Dasari S, Vrana JA, et al. Congophilic fibrillary glomerulonephritis: a case series[J]. Am J Kidney Dis, 2018, 72(3): 325-336. doi: S0272-6386(18)30604-8 pmid: 29866458
[27]	梁少姗, 杨帆, 曾彩虹. 刚果红阳性的纤维性肾小球肾炎[J]. 肾脏病与透析肾移植杂志, 2019, 28(2): 189-193.
	Liang SS, Yang F, Zeng CH. Fibrous glomerulonephritis with Congo red positivity[J]. Shenzangbing Yu Touxi Shenyizhi Zazhi, 2019, 28(2): 189-193.
[28]	周玉超, 姜玲, 程震. 纤维性肾小球肾炎两例及文献复习[J]. 中华肾脏病杂志, 2021, 37(8): 673-676. doi: 10.3760/cma.j.cn441217-20200518-00100
	Zhou YC, Jiang L, Cheng Z. Two cases of fibrillary glomerulonephritis and a literature review[J]. Zhonghua Shenzangbing Zazhi, 2021, 37(8): 673-676.
[29]	Rosenstock JL, Markowitz GS, Valeri AM, et al. Fibrillary and immunotactoid glomerulonephritis: Distinct entities with different clinical and pathologic features[J]. Kidney Int, 2003, 63(4): 1450-1461. doi: 10.1046/j.1523-1755.2003.00853.x pmid: 12631361
[30]	张宏文, 崔洁媛, 苏白鸽, 等. 儿童纤维性肾小球肾炎1例报告并文献复习[J]. 临床儿科杂志, 2017, 35(9): 687-690.
	Zhang HW, Cui JY, Su BG, et al. A case report of fibrillary glomerulonephritis in a child and a literature review[J]. Linchuang Erke Zazhi, 2017, 35(9): 687-690.
[31]	Menon S, Zeng X, Valentini R. Fibrillary glome-rulonephritis and renal failure in a child with systemic lupus erythematosus[J]. PEDIATR NEPHROL, 2009, 24 (8): 1577-1581. doi: 10.1007/s00467-009-1168-z
[32]	徐秋郁, 文煜冰, 崔皓园, 等. 临床进展相对缓慢的纤维性肾小球肾炎1例[J]. 中华内科杂志, 2024, 63(4): 412-415.
	Xu QY, Wen YB, Cui HY, et al. A case of fibrillary glomerulonephritis with relatively slow clinical progression[J]. Zhonghua Neike Zazhi, 2024, 63(4): 412-415.
[33]	Nasr SH, Dasari S, Lieske JC, et al. Serum levels of DNAJB9 are elevated in fibrillary glomerulonephritis patients[J]. Kidney Int, 2019, 95(5): 1269-1272. doi: 10.1016/j.kint.2019.01.024
[34]	Cohen AWS, Vilayur E. Fibrillary and immunotactoid glomerulopathies in the Hunter region: a retrospective cohort study[J]. Intern Med J, 2023, 53(10): 1837-1845. doi: 10.1111/imj.v53.10
[35]	孙小玲, 张芳成, 肖艺, 等. 免疫触须样肾小球病的病理形态观察及鉴别诊断[J]. 中华病理学杂志, 2016, 45(10): 703-706.
	Sun XL, Zhang FC, Xiao Y, et al. Pathological morphology observation and differential diagnosis of immune tuft-like glomerulopathy[J]. Zhonghua Binglixue Zazhi, 2016, 45(10): 703-706.
[36]	SSekulic M, Nasr SH, Grande JP, et al. Histologic regression of fibrillary glomerulonephritis: the first report of biopsy-proven spontaneous resolution of disease[J]. Clin Kidney J, 2017, 10(6): 738-741. doi: 10.1093/ckj/sfx045 pmid: 29225801
[37]	Nasr SH, Valeri AM, Cornell LD, et al. Fibrillary glomerulonephritis: a report of 66 cases from a single institution[J]. Clin J Am Soc Nephro, 2011, 6(4): 775-784. doi: 10.2215/CJN.08300910 pmid: 21441134
[38]	Javaugue V, Karras A, Glowacki F, et al. Long-term kidney disease outcomes in fibrillary glomerulonephritis: a case series of 27 patients[J]. Am J Kidney Dis, 2013, 62 (4): 679-690. doi: 10.1053/j.ajkd.2013.03.031 pmid: 23759297
[39]	Maroz N, Reuben S, Nadasdy T. Treatment of fibrillary glomerulonephritis with use of repository corticotropin injections[J]. Clin Kidney J, 2018, 11(6): 788-790. doi: 10.1093/ckj/sfy009 pmid: 30524713
[40]	Madan A, Mijovic-Das S, Stankovic A, et al. Acthar gel in the treatment of nephrotic syndrome: a multicenter retrospective case series[J]. BMC Nephrol, 2016, 17: 37. doi: 10.1186/s12882-016-0241-7 pmid: 27036111
[41]	刘海玉, 赵德安. 儿童微小病变型肾病综合征免疫学机制研究进展[J]. 现代临床医学, 2020, 46(2): 154-157.
	Liu HY, Zhao DA. Advances in the study of immunological mechanisms of minimal change nephrotic syndrome in children[J]. Xiandai Linchuang Yixue, 2020, 46(2): 154-157.
[42]	刘海玉, 赵德安, 贾妮旦, 等. 原发性肾病综合征患儿外周血单个核细胞中Toll样受体-2、-4、-6表达与血清IgG、IgM相关性分析[J]. 中华实用儿科临床杂志, 2019, 34(22): 1728-1731.
	Liu HY, Zhao DA, Jia ND, et al. Correlation analysis of Toll-like receptor-2, -4, -6 expression in peripheral blood mononuclear cells and serum IgG, IgM in children with primary nephrotic syndrome[J]. Zhonghua Shiyong Erke Linchuang Zazhi, 2019, 34(22): 1728-1731.
[43]	中华医学会肾脏病学分会专家组. 利妥昔单抗在肾小球肾炎中应用的专家共识[J]. 中华肾脏病杂志, 2022, 38(2): 151-160. doi: 10.3760/cma.j.cn441217-20210615-00023
	Expert Group of Nephrology Branch of Chinese Medical Association. Expert consensus on the application of rituximab in glomerulonephritis[J]. Zhonghua Shenzangbing Zazhi, 2022, 38(2): 151-160.
[44]	Collins M, Navaneethan SD, Chung M, et al. Rituximab treatment of fibrillary glomerulonephritis[J]. Am J Kidney Dis, 2008, 52(6): 1158-1162. doi: 10.1053/j.ajkd.2008.07.011 pmid: 18823685
[45]	Erickson SB, Zand L, Nasr SH, et al. Treatment of fibrillary glomerulonephritis with rituximab: a 12-month pilot study[J]. Nephrol Dial Transplant, 2021, 36(1): 104-110. doi: 10.1093/ndt/gfaa065
[46]	Payan Schober F, Jobson MA, Poulton CJ, et al. Clinical features and outcomes of a racially diverse population with fibrillary glomerulonephritis[J]. Am J Nephrol, 2017, 45(3): 248-256. doi: 10.1159/000455390 pmid: 28161700
[47]	Hogan J, Restivo M, Canetta PA, et al. Rituximab treatment for fibrillary glomerulonephritis[J]. Nephrol Dial Transplant, 2014, 29(10): 1925-1931. doi: 10.1093/ndt/gfu189
[48]	Alexander MP, Dasari S, Vrana JA, et al. Congophilic fibrillary glomerulonephritis: a case series[J]. Am J Kidney Dis, 2018, 72(3): 325-336. doi: S0272-6386(18)30604-8 pmid: 29866458
[49]	Andeen NK, Troxell ML, Riazy M, et al. Fibrillary glomerulonephritis: clinicopathologic features and atypical cases from a multi-institutional cohort[J]. Clin J Am Soc Nephrol, 2019, 14(12): 1741-1750. doi: 10.2215/CJN.03870319
[50]	Sekulic M, Nasr SH, Grande JP, et al. Histologic regression of fibrillary glomerulonephritis: the first report of biopsy-proven spontaneous resolution of disease[J]. Clin Kidney J, 2017, 10(6): 738-741. doi: 10.1093/ckj/sfx045 pmid: 29225801

Advances in pathogenesis, diagnosis and treatment of fibrillary glomerulonephritis

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[7]	LI Wanghui, SU Dayong, QIANG Ruixue, et al. Steroid-resistant nephrotic syndrome caused by PLCE1 gene mutation: a case report and literature review [J]. Journal of Clinical Pediatrics, 2020, 38(6): 422-.
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[9]	CAI Xiaoyi, DENG Huiying, CHEN Ye, et al. A retrospective cohort study on gene variation and clinical features of steroid resistant nephrotic syndrome in children [J]. Journal of Clinical Pediatrics, 2020, 38(10): 740-.
[10]	FU Yanhong, MA Liyan, WANG Lian, et al. Relevant factors of recurrent primary nephrotic syndrome in children in Ningxia [J]. Journal of Clinical Pediatrics, 2019, 37(9): 652-.
[11]	CHEN Yanyun, ZHANG Xiaolei. Genetic mutation in congenital nephrotic syndrome Finnish type: a case report and literature review　 [J]. Journal of Clinical Pediatrics, 2019, 37(6): 445-.
[12]	LIANG Fangfang, PENG Cheng, HUANG Yanyan, et al. The clinical, pathological and pathogenic gene characteristics of Frasier syndrome: a case report [J]. Journal of Clinical Pediatrics, 2019, 37(3): 215-.
[13]	GAO Jinzhi, CHEN Ling . Congenital nephrotic syndrome of the Finnish type: a case report with literature review [J]. , 2018, 36(9): 670-.
[14]	WU Heyan, GAO Chunlin, LU Mei. Progress in epidemiology of focal segmental glomerulosclerosis [J]. , 2018, 36(9): 716-.
[15]	LIU Xiaoyu, TANG Zezhong, WANG Fang, ZHANG Hongwen, CHEN Zhihui, YAO Yong, DING Jie . Clinical characteristics, treatment and follow-up of Cockayne syndrome with renal involvement [J]. , 2018, 36(6): 424-.