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Fibrillary glomerulonephritis

MedGen UID:
473006
•Concept ID:
C0268749
•
Disease or Syndrome
Synonyms: Amyloid-like glomerulopathy; Fibrillary Glomerulonephritis; Non-Amyloid Fibrillary Glomerulonephritis
SNOMED CT: Fibrillary glomerulonephritis (73305009); Amyloid-like glomerulopathy (73305009)

Definition

Glomerulonephritis characterized by the presence of Congo-red negative microfibrils in the mesangium and capillary walls of the glomeruli. It is associated with marked proteinuria and leads to progressive renal failure. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFibrillary glomerulonephritis

Professional guidelines

PubMed

Treatment of fibrillary glomerulonephritis with rituximab: a 12-month pilot study.
Erickson SB, Zand L, Nasr SH, Alexander MP, Leung N, Drosou ME, Fervenza FC
Nephrol Dial Transplant 2021 Jan 1;36(1):104-110. doi: 10.1093/ndt/gfaa065. PMID: 32617582
Rituximab treatment for fibrillary glomerulonephritis.
Hogan J, Restivo M, Canetta PA, Herlitz LC, Radhakrishnan J, Appel GB, Bomback AS
Nephrol Dial Transplant 2014 Oct;29(10):1925-31. Epub 2014 May 27 doi: 10.1093/ndt/gfu189. PMID: 24867652

Recent clinical studies

Etiology

Clinicopathological characteristics and outcome of patients with fibrillary glomerulonephritis: DNAJB9 is a valuable histologic marker.
Liang S, Chen D, Liang D, Xu F, Zhang M, Yang F, Zhu X, Li P, Zeng C
J Nephrol 2021 Jun;34(3):883-892. Epub 2020 Jun 18 doi: 10.1007/s40620-020-00783-4. PMID: 32557205
Fibrillary Glomerulonephritis: Clinicopathologic Features and Atypical Cases from a Multi-Institutional Cohort.
Andeen NK, Troxell ML, Riazy M, Avasare RS, Lapasia J, Jefferson JA, Akilesh S, Najafian B, Nicosia RF, Alpers CE, Smith KD
Clin J Am Soc Nephrol 2019 Dec 6;14(12):1741-1750. Epub 2019 Nov 4 doi: 10.2215/CJN.03870319. PMID: 31685544Free PMC Article
Dysproteinemia and the Kidney: Core Curriculum 2019.
Hogan JJ, Alexander MP, Leung N
Am J Kidney Dis 2019 Dec;74(6):822-836. Epub 2019 Jul 19 doi: 10.1053/j.ajkd.2019.04.029. PMID: 31331759
Congophilic Fibrillary Glomerulonephritis: A Case Series.
Alexander MP, Dasari S, Vrana JA, Riopel J, Valeri AM, Markowitz GS, Hever A, Bijol V, Larsen CP, Cornell LD, Fidler ME, Said SM, Sethi S, Herrera Hernandez LP, Grande JP, Erickson SB, Fervenza FC, Leung N, Kurtin PJ, Nasr SH
Am J Kidney Dis 2018 Sep;72(3):325-336. Epub 2018 Jun 14 doi: 10.1053/j.ajkd.2018.03.017. PMID: 29866458
IgG Subclass Staining in Routine Renal Biopsy Material.
Hemminger J, Nadasdy G, Satoskar A, Brodsky SV, Nadasdy T
Am J Surg Pathol 2016 May;40(5):617-26. doi: 10.1097/PAS.0000000000000605. PMID: 26848798

Diagnosis

DNA J homolog subfamily B member 9 and other advances in fibrillary glomerulonephritis.
Andeen NK, Avasare RS
Curr Opin Nephrol Hypertens 2021 May 1;30(3):294-302. doi: 10.1097/MNH.0000000000000706. PMID: 33767057
Fibrillary Glomerulonephritis and DnaJ Homolog Subfamily B Member 9 (DNAJB9).
Klomjit N, Alexander MP, Zand L
Kidney360 2020 Sep 24;1(9):1002-1013. Epub 2020 Jul 8 doi: 10.34067/KID.0002532020. PMID: 35369552Free PMC Article
Fibrillary Glomerulonephritis: Clinicopathologic Features and Atypical Cases from a Multi-Institutional Cohort.
Andeen NK, Troxell ML, Riazy M, Avasare RS, Lapasia J, Jefferson JA, Akilesh S, Najafian B, Nicosia RF, Alpers CE, Smith KD
Clin J Am Soc Nephrol 2019 Dec 6;14(12):1741-1750. Epub 2019 Nov 4 doi: 10.2215/CJN.03870319. PMID: 31685544Free PMC Article
Dysproteinemia and the Kidney: Core Curriculum 2019.
Hogan JJ, Alexander MP, Leung N
Am J Kidney Dis 2019 Dec;74(6):822-836. Epub 2019 Jul 19 doi: 10.1053/j.ajkd.2019.04.029. PMID: 31331759
New developments in the diagnosis of fibrillary glomerulonephritis.
Nasr SH, Fogo AB
Kidney Int 2019 Sep;96(3):581-592. Epub 2019 Apr 9 doi: 10.1016/j.kint.2019.03.021. PMID: 31227146

Therapy

Fibrillary Glomerulonephritis and DnaJ Homolog Subfamily B Member 9 (DNAJB9).
Klomjit N, Alexander MP, Zand L
Kidney360 2020 Sep 24;1(9):1002-1013. Epub 2020 Jul 8 doi: 10.34067/KID.0002532020. PMID: 35369552Free PMC Article
Fibrillary Glomerulonephritis: Clinicopathologic Features and Atypical Cases from a Multi-Institutional Cohort.
Andeen NK, Troxell ML, Riazy M, Avasare RS, Lapasia J, Jefferson JA, Akilesh S, Najafian B, Nicosia RF, Alpers CE, Smith KD
Clin J Am Soc Nephrol 2019 Dec 6;14(12):1741-1750. Epub 2019 Nov 4 doi: 10.2215/CJN.03870319. PMID: 31685544Free PMC Article
Dysproteinemia and the Kidney: Core Curriculum 2019.
Hogan JJ, Alexander MP, Leung N
Am J Kidney Dis 2019 Dec;74(6):822-836. Epub 2019 Jul 19 doi: 10.1053/j.ajkd.2019.04.029. PMID: 31331759
New developments in the diagnosis of fibrillary glomerulonephritis.
Nasr SH, Fogo AB
Kidney Int 2019 Sep;96(3):581-592. Epub 2019 Apr 9 doi: 10.1016/j.kint.2019.03.021. PMID: 31227146
Hepatitis C virus associated glomerulopathies.
Ozkok A, Yildiz A
World J Gastroenterol 2014 Jun 28;20(24):7544-54. doi: 10.3748/wjg.v20.i24.7544. PMID: 24976695Free PMC Article

Prognosis

Fibrillary Glomerulonephritis and DnaJ Homolog Subfamily B Member 9 (DNAJB9).
Klomjit N, Alexander MP, Zand L
Kidney360 2020 Sep 24;1(9):1002-1013. Epub 2020 Jul 8 doi: 10.34067/KID.0002532020. PMID: 35369552Free PMC Article
Fibrillary Glomerulonephritis: Clinicopathologic Features and Atypical Cases from a Multi-Institutional Cohort.
Andeen NK, Troxell ML, Riazy M, Avasare RS, Lapasia J, Jefferson JA, Akilesh S, Najafian B, Nicosia RF, Alpers CE, Smith KD
Clin J Am Soc Nephrol 2019 Dec 6;14(12):1741-1750. Epub 2019 Nov 4 doi: 10.2215/CJN.03870319. PMID: 31685544Free PMC Article
New developments in the diagnosis of fibrillary glomerulonephritis.
Nasr SH, Fogo AB
Kidney Int 2019 Sep;96(3):581-592. Epub 2019 Apr 9 doi: 10.1016/j.kint.2019.03.021. PMID: 31227146
Congophilic Fibrillary Glomerulonephritis: A Case Series.
Alexander MP, Dasari S, Vrana JA, Riopel J, Valeri AM, Markowitz GS, Hever A, Bijol V, Larsen CP, Cornell LD, Fidler ME, Said SM, Sethi S, Herrera Hernandez LP, Grande JP, Erickson SB, Fervenza FC, Leung N, Kurtin PJ, Nasr SH
Am J Kidney Dis 2018 Sep;72(3):325-336. Epub 2018 Jun 14 doi: 10.1053/j.ajkd.2018.03.017. PMID: 29866458
IgG Subclass Staining in Routine Renal Biopsy Material.
Hemminger J, Nadasdy G, Satoskar A, Brodsky SV, Nadasdy T
Am J Surg Pathol 2016 May;40(5):617-26. doi: 10.1097/PAS.0000000000000605. PMID: 26848798

Clinical prediction guides

Genetic or pharmacologic Nrf2 activation increases proteinuria in chronic kidney disease in mice.
Rush BM, Bondi CD, Stocker SD, Barry KM, Small SA, Ong J, Jobbagy S, Stolz DB, Bastacky SI, Chartoumpekis DV, Kensler TW, Tan RJ
Kidney Int 2021 Jan;99(1):102-116. Epub 2020 Aug 18 doi: 10.1016/j.kint.2020.07.036. PMID: 32818518Free PMC Article
Clinicopathological characteristics and outcome of patients with fibrillary glomerulonephritis: DNAJB9 is a valuable histologic marker.
Liang S, Chen D, Liang D, Xu F, Zhang M, Yang F, Zhu X, Li P, Zeng C
J Nephrol 2021 Jun;34(3):883-892. Epub 2020 Jun 18 doi: 10.1007/s40620-020-00783-4. PMID: 32557205
Fibrillary Glomerulonephritis: Clinicopathologic Features and Atypical Cases from a Multi-Institutional Cohort.
Andeen NK, Troxell ML, Riazy M, Avasare RS, Lapasia J, Jefferson JA, Akilesh S, Najafian B, Nicosia RF, Alpers CE, Smith KD
Clin J Am Soc Nephrol 2019 Dec 6;14(12):1741-1750. Epub 2019 Nov 4 doi: 10.2215/CJN.03870319. PMID: 31685544Free PMC Article
Clinical Features and Outcomes of a Racially Diverse Population with Fibrillary Glomerulonephritis.
Payan Schober F, Jobson MA, Poulton CJ, Singh HK, Nickeleit V, Falk RJ, Jennette JC, Nachman PH, Pendergraft Iii WF
Am J Nephrol 2017;45(3):248-256. Epub 2017 Feb 4 doi: 10.1159/000455390. PMID: 28161700Free PMC Article
IgG Subclass Staining in Routine Renal Biopsy Material.
Hemminger J, Nadasdy G, Satoskar A, Brodsky SV, Nadasdy T
Am J Surg Pathol 2016 May;40(5):617-26. doi: 10.1097/PAS.0000000000000605. PMID: 26848798

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