Anti-PLA2R Antibody Development During NELL1-Associated Membranous Glomerulonephritis Treatment: A Case Report
A Japanese man in his early 70s was referred to our hospital because of massive proteinuria. Analysis of his kidney biopsy demonstrated glomerular subepithelial immune deposits containing immunoglobulin (Ig)G, which was dominant for the IgG1 subclass. Immunoperoxidase staining for neural epidermal g...
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| Format: | Article |
| Language: | English |
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Elsevier
2023-05-01
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| Series: | Kidney Medicine |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2590059523000341 |
| _version_ | 1797845012259536896 |
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| author | Dan Inoue Takahiro Uchida Shuuhei Komatsu Kentaro Sugisaki Muneharu Yamada Hodaka Ogawa Akihiro Ishizu Takashi Oda |
| author_facet | Dan Inoue Takahiro Uchida Shuuhei Komatsu Kentaro Sugisaki Muneharu Yamada Hodaka Ogawa Akihiro Ishizu Takashi Oda |
| author_sort | Dan Inoue |
| collection | DOAJ |
| description | A Japanese man in his early 70s was referred to our hospital because of massive proteinuria. Analysis of his kidney biopsy demonstrated glomerular subepithelial immune deposits containing immunoglobulin (Ig)G, which was dominant for the IgG1 subclass. Immunoperoxidase staining for neural epidermal growth factor-like 1 protein (NELL1) was positive on the glomerular capillary walls, whereas neither serum anti-phospholipase A2 receptor (PLA2R) antibodies nor immunofluorescence staining for PLA2R was positive. Detailed investigation revealed no associated conditions, including underlying malignancies, and thus he was diagnosed as having NELL1-associated idiopathic membranous nephropathy (MN). The patient was treated with steroids, which substantially improved his nephrotic syndrome. Interestingly, serum anti-NELL1 as well as anti-PLA2R antibodies became positive during his clinical course. Serology-based approaches are currently proposed for the treatment of patients suspected of having MN; however, an accurate diagnosis of the present patient would have been difficult if such an approach was performed only at a later phase of the disease. Several target antigens for the glomerular immune deposits observed in patients with MN have recently been identified, and dual positivity of antibodies to these antigens reportedly occurs in some patients. Further accumulation and analyses of such patients are needed to establish more appropriate diagnostic approaches for MN. |
| first_indexed | 2024-04-09T17:32:42Z |
| format | Article |
| id | doaj.art-9dcfd485dbe24dfc9821f1d44731fac8 |
| institution | Directory Open Access Journal |
| issn | 2590-0595 |
| language | English |
| last_indexed | 2024-04-09T17:32:42Z |
| publishDate | 2023-05-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Kidney Medicine |
| spelling | doaj.art-9dcfd485dbe24dfc9821f1d44731fac82023-04-18T04:09:08ZengElsevierKidney Medicine2590-05952023-05-0155100625Anti-PLA2R Antibody Development During NELL1-Associated Membranous Glomerulonephritis Treatment: A Case ReportDan Inoue0Takahiro Uchida1Shuuhei Komatsu2Kentaro Sugisaki3Muneharu Yamada4Hodaka Ogawa5Akihiro Ishizu6Takashi Oda7Department of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, Tokyo, JapanDepartment of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, Tokyo, Japan; Address for Correspondence: Takahiro Uchida, MD, PhD, Department of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo 193-0998, Japan.Department of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, Tokyo, JapanDepartment of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, Tokyo, JapanDepartment of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, Tokyo, JapanDepartment of Medical Laboratory Science, Faculty of Health Sciences, Hokkaido University, Sapporo, Hokkaido, JapanDepartment of Medical Laboratory Science, Faculty of Health Sciences, Hokkaido University, Sapporo, Hokkaido, JapanDepartment of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, Tokyo, JapanA Japanese man in his early 70s was referred to our hospital because of massive proteinuria. Analysis of his kidney biopsy demonstrated glomerular subepithelial immune deposits containing immunoglobulin (Ig)G, which was dominant for the IgG1 subclass. Immunoperoxidase staining for neural epidermal growth factor-like 1 protein (NELL1) was positive on the glomerular capillary walls, whereas neither serum anti-phospholipase A2 receptor (PLA2R) antibodies nor immunofluorescence staining for PLA2R was positive. Detailed investigation revealed no associated conditions, including underlying malignancies, and thus he was diagnosed as having NELL1-associated idiopathic membranous nephropathy (MN). The patient was treated with steroids, which substantially improved his nephrotic syndrome. Interestingly, serum anti-NELL1 as well as anti-PLA2R antibodies became positive during his clinical course. Serology-based approaches are currently proposed for the treatment of patients suspected of having MN; however, an accurate diagnosis of the present patient would have been difficult if such an approach was performed only at a later phase of the disease. Several target antigens for the glomerular immune deposits observed in patients with MN have recently been identified, and dual positivity of antibodies to these antigens reportedly occurs in some patients. Further accumulation and analyses of such patients are needed to establish more appropriate diagnostic approaches for MN.http://www.sciencedirect.com/science/article/pii/S2590059523000341IgG subclassmembranous nephropathyNELL1nephrotic syndromePLA2R |
| spellingShingle | Dan Inoue Takahiro Uchida Shuuhei Komatsu Kentaro Sugisaki Muneharu Yamada Hodaka Ogawa Akihiro Ishizu Takashi Oda Anti-PLA2R Antibody Development During NELL1-Associated Membranous Glomerulonephritis Treatment: A Case Report Kidney Medicine IgG subclass membranous nephropathy NELL1 nephrotic syndrome PLA2R |
| title | Anti-PLA2R Antibody Development During NELL1-Associated Membranous Glomerulonephritis Treatment: A Case Report |
| title_full | Anti-PLA2R Antibody Development During NELL1-Associated Membranous Glomerulonephritis Treatment: A Case Report |
| title_fullStr | Anti-PLA2R Antibody Development During NELL1-Associated Membranous Glomerulonephritis Treatment: A Case Report |
| title_full_unstemmed | Anti-PLA2R Antibody Development During NELL1-Associated Membranous Glomerulonephritis Treatment: A Case Report |
| title_short | Anti-PLA2R Antibody Development During NELL1-Associated Membranous Glomerulonephritis Treatment: A Case Report |
| title_sort | anti pla2r antibody development during nell1 associated membranous glomerulonephritis treatment a case report |
| topic | IgG subclass membranous nephropathy NELL1 nephrotic syndrome PLA2R |
| url | http://www.sciencedirect.com/science/article/pii/S2590059523000341 |
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