Fibrillary Glomerulonephritis: An Update
Fibrillary glomerulonephritis (FGN) is a rare proliferative form of glomerular disease characterized by randomly oriented fibrillar deposits with a mean diameter of 20 nm. By immunofluorescence (IF), the deposits stain for IgG, C3, and κ and λ light chains, suggesting that the fibrils may be compose...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Elsevier
2019-07-01
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| Series: | Kidney International Reports |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2468024919301603 |
| _version_ | 1811226306261549056 |
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| author | Jordan L. Rosenstock Glen S. Markowitz |
| author_facet | Jordan L. Rosenstock Glen S. Markowitz |
| author_sort | Jordan L. Rosenstock |
| collection | DOAJ |
| description | Fibrillary glomerulonephritis (FGN) is a rare proliferative form of glomerular disease characterized by randomly oriented fibrillar deposits with a mean diameter of 20 nm. By immunofluorescence (IF), the deposits stain for IgG, C3, and κ and λ light chains, suggesting that the fibrils may be composed of antigen-antibody immune complexes. A recent major advance in our understanding of the pathogenesis of FGN resulted from the discovery that a major component of the fibrils is DNA-J heat-shock protein family member B9 (DNAJB9), and immunohistochemical staining for DNAJB9 now makes it possible to diagnose FGN in the absence of ultrastructural evaluation. FGN has a poor prognosis, treatment options are currently limited, and transplant recurrence is not uncommon. Keywords: fibrillary glomerulonephritis, DNAJB9 |
| first_indexed | 2024-04-12T09:22:59Z |
| format | Article |
| id | doaj.art-b3610ffeded14d18b375f0f77a1587af |
| institution | Directory Open Access Journal |
| issn | 2468-0249 |
| language | English |
| last_indexed | 2024-04-12T09:22:59Z |
| publishDate | 2019-07-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Kidney International Reports |
| spelling | doaj.art-b3610ffeded14d18b375f0f77a1587af2022-12-22T03:38:34ZengElsevierKidney International Reports2468-02492019-07-0147917922Fibrillary Glomerulonephritis: An UpdateJordan L. Rosenstock0Glen S. Markowitz1Division of Nephrology, Lenox Hill Hospital, Hofstra Northwell School of Medicine, New York, New York, USA; Correspondence: Jordan L. Rosenstock, Division of Nephrology, Lenox Hill Hospital, Northwell Health, 130 East 77th Street, 5th Floor, New York, NY 10075, USA.Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USAFibrillary glomerulonephritis (FGN) is a rare proliferative form of glomerular disease characterized by randomly oriented fibrillar deposits with a mean diameter of 20 nm. By immunofluorescence (IF), the deposits stain for IgG, C3, and κ and λ light chains, suggesting that the fibrils may be composed of antigen-antibody immune complexes. A recent major advance in our understanding of the pathogenesis of FGN resulted from the discovery that a major component of the fibrils is DNA-J heat-shock protein family member B9 (DNAJB9), and immunohistochemical staining for DNAJB9 now makes it possible to diagnose FGN in the absence of ultrastructural evaluation. FGN has a poor prognosis, treatment options are currently limited, and transplant recurrence is not uncommon. Keywords: fibrillary glomerulonephritis, DNAJB9http://www.sciencedirect.com/science/article/pii/S2468024919301603 |
| spellingShingle | Jordan L. Rosenstock Glen S. Markowitz Fibrillary Glomerulonephritis: An Update Kidney International Reports |
| title | Fibrillary Glomerulonephritis: An Update |
| title_full | Fibrillary Glomerulonephritis: An Update |
| title_fullStr | Fibrillary Glomerulonephritis: An Update |
| title_full_unstemmed | Fibrillary Glomerulonephritis: An Update |
| title_short | Fibrillary Glomerulonephritis: An Update |
| title_sort | fibrillary glomerulonephritis an update |
| url | http://www.sciencedirect.com/science/article/pii/S2468024919301603 |
| work_keys_str_mv | AT jordanlrosenstock fibrillaryglomerulonephritisanupdate AT glensmarkowitz fibrillaryglomerulonephritisanupdate |