Rare case of exostosin 1/exostosin 2-related membranous lupus nephritis concomitant with dual ANCA- and anti-GBM antibody-associated crescentic glomerulonephritis effectively diagnosed by mass spectrometry: a case report
Abstract Background Recent developments in mass spectrometry (MS) have revealed target antigens for membranous nephropathy (MN), including phospholipase A2 receptor and exostosin 1/exostosin 2 (EXT1/2). EXT1/2 are known antigens of autoimmune disease-related MN, especially membranous lupus nephritis...
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| Format: | Article |
| Language: | English |
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BMC
2023-07-01
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| Series: | BMC Nephrology |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12882-023-03268-1 |
| _version_ | 1797769625407062016 |
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| author | Takuya Yamazaki Haruka Takahashi Kazuhiro Takeuchi Emi Sakamoto Kenta Tominaga Syun Sakurabayashi Tetsuya Abe Takashi Sano Yukihiro Wada Naomi Kuwahara Akira Shimizu Yasuo Takeuchi |
| author_facet | Takuya Yamazaki Haruka Takahashi Kazuhiro Takeuchi Emi Sakamoto Kenta Tominaga Syun Sakurabayashi Tetsuya Abe Takashi Sano Yukihiro Wada Naomi Kuwahara Akira Shimizu Yasuo Takeuchi |
| author_sort | Takuya Yamazaki |
| collection | DOAJ |
| description | Abstract Background Recent developments in mass spectrometry (MS) have revealed target antigens for membranous nephropathy (MN), including phospholipase A2 receptor and exostosin 1/exostosin 2 (EXT1/2). EXT1/2 are known antigens of autoimmune disease-related MN, especially membranous lupus nephritis. We describe the case of an elderly man who developed nephrotic syndrome followed by progressive renal dysfunction. Case presentation A 78-year-old man presented with rapidly progressive renal dysfunction with proteinuria and hematuria. Three years previously, he had developed leg edema but did not receive any treatment. Laboratory tests showed elevated anti-nuclear antibody (Ab), anti-dsDNA Ab titer, and hypocomplementemia, indicating systemic lupus erythematous. Myeloperoxidase anti-neutrophil cytoplasmic Ab (ANCA) and anti-glomerular basement membrane (GBM) Ab were also detected. The renal pathologic findings were compatible with crescentic glomerulonephritis (GN), whereas non-crescentic glomeruli exhibited MN without remarkable endocapillary or mesangial proliferative change. Immunofluorescence microscopy revealed glomerular IgG, C3, and C1q deposition. All IgG subclasses were positive in glomeruli. Anti-PLA2R Ab in serum was negative. MS analysis was performed to detect the antigens of MN, and EXT1/2 was detected in glomeruli. Therefore, we reached a diagnosis of membranous lupus nephritis concurrent with both ANCA-associated vasculitis and anti-GBM-GN. The simultaneous occurrence of these three diseases is extremely rare. Conclusions This is the first report of EXT1/2-related membranous lupus nephritis concurrent with ANCA-associated vasculitis and anti-GBM-GN. This case demonstrates the usefulness of MS in diagnosing complicated cases of MN. |
| first_indexed | 2024-03-12T21:10:47Z |
| format | Article |
| id | doaj.art-351c070a89044d8bbe6c729b2c87547f |
| institution | Directory Open Access Journal |
| issn | 1471-2369 |
| language | English |
| last_indexed | 2024-03-12T21:10:47Z |
| publishDate | 2023-07-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Nephrology |
| spelling | doaj.art-351c070a89044d8bbe6c729b2c87547f2023-07-30T11:09:49ZengBMCBMC Nephrology1471-23692023-07-012411610.1186/s12882-023-03268-1Rare case of exostosin 1/exostosin 2-related membranous lupus nephritis concomitant with dual ANCA- and anti-GBM antibody-associated crescentic glomerulonephritis effectively diagnosed by mass spectrometry: a case reportTakuya Yamazaki0Haruka Takahashi1Kazuhiro Takeuchi2Emi Sakamoto3Kenta Tominaga4Syun Sakurabayashi5Tetsuya Abe6Takashi Sano7Yukihiro Wada8Naomi Kuwahara9Akira Shimizu10Yasuo Takeuchi11Department of Nephrology, Kitasato University School of MedicineDepartment of Nephrology, Kitasato University School of MedicineDepartment of Nephrology, Kitasato University School of MedicineDepartment of Analytic Human Pathology, Nippon Medical SchoolDepartment of Analytic Human Pathology, Nippon Medical SchoolDepartment of Nephrology, Kitasato University School of MedicineDepartment of Nephrology, Kitasato University School of MedicineAihara HospitalDepartment of Nephrology, Kitasato University School of MedicineDepartment of Analytic Human Pathology, Nippon Medical SchoolDepartment of Analytic Human Pathology, Nippon Medical SchoolDepartment of Nephrology, Kitasato University School of MedicineAbstract Background Recent developments in mass spectrometry (MS) have revealed target antigens for membranous nephropathy (MN), including phospholipase A2 receptor and exostosin 1/exostosin 2 (EXT1/2). EXT1/2 are known antigens of autoimmune disease-related MN, especially membranous lupus nephritis. We describe the case of an elderly man who developed nephrotic syndrome followed by progressive renal dysfunction. Case presentation A 78-year-old man presented with rapidly progressive renal dysfunction with proteinuria and hematuria. Three years previously, he had developed leg edema but did not receive any treatment. Laboratory tests showed elevated anti-nuclear antibody (Ab), anti-dsDNA Ab titer, and hypocomplementemia, indicating systemic lupus erythematous. Myeloperoxidase anti-neutrophil cytoplasmic Ab (ANCA) and anti-glomerular basement membrane (GBM) Ab were also detected. The renal pathologic findings were compatible with crescentic glomerulonephritis (GN), whereas non-crescentic glomeruli exhibited MN without remarkable endocapillary or mesangial proliferative change. Immunofluorescence microscopy revealed glomerular IgG, C3, and C1q deposition. All IgG subclasses were positive in glomeruli. Anti-PLA2R Ab in serum was negative. MS analysis was performed to detect the antigens of MN, and EXT1/2 was detected in glomeruli. Therefore, we reached a diagnosis of membranous lupus nephritis concurrent with both ANCA-associated vasculitis and anti-GBM-GN. The simultaneous occurrence of these three diseases is extremely rare. Conclusions This is the first report of EXT1/2-related membranous lupus nephritis concurrent with ANCA-associated vasculitis and anti-GBM-GN. This case demonstrates the usefulness of MS in diagnosing complicated cases of MN.https://doi.org/10.1186/s12882-023-03268-1Lupus nephritisANCA-associated vasculitisAnti-GBM glomerulonephritisMass spectrometryExostosin ½ |
| spellingShingle | Takuya Yamazaki Haruka Takahashi Kazuhiro Takeuchi Emi Sakamoto Kenta Tominaga Syun Sakurabayashi Tetsuya Abe Takashi Sano Yukihiro Wada Naomi Kuwahara Akira Shimizu Yasuo Takeuchi Rare case of exostosin 1/exostosin 2-related membranous lupus nephritis concomitant with dual ANCA- and anti-GBM antibody-associated crescentic glomerulonephritis effectively diagnosed by mass spectrometry: a case report BMC Nephrology Lupus nephritis ANCA-associated vasculitis Anti-GBM glomerulonephritis Mass spectrometry Exostosin ½ |
| title | Rare case of exostosin 1/exostosin 2-related membranous lupus nephritis concomitant with dual ANCA- and anti-GBM antibody-associated crescentic glomerulonephritis effectively diagnosed by mass spectrometry: a case report |
| title_full | Rare case of exostosin 1/exostosin 2-related membranous lupus nephritis concomitant with dual ANCA- and anti-GBM antibody-associated crescentic glomerulonephritis effectively diagnosed by mass spectrometry: a case report |
| title_fullStr | Rare case of exostosin 1/exostosin 2-related membranous lupus nephritis concomitant with dual ANCA- and anti-GBM antibody-associated crescentic glomerulonephritis effectively diagnosed by mass spectrometry: a case report |
| title_full_unstemmed | Rare case of exostosin 1/exostosin 2-related membranous lupus nephritis concomitant with dual ANCA- and anti-GBM antibody-associated crescentic glomerulonephritis effectively diagnosed by mass spectrometry: a case report |
| title_short | Rare case of exostosin 1/exostosin 2-related membranous lupus nephritis concomitant with dual ANCA- and anti-GBM antibody-associated crescentic glomerulonephritis effectively diagnosed by mass spectrometry: a case report |
| title_sort | rare case of exostosin 1 exostosin 2 related membranous lupus nephritis concomitant with dual anca and anti gbm antibody associated crescentic glomerulonephritis effectively diagnosed by mass spectrometry a case report |
| topic | Lupus nephritis ANCA-associated vasculitis Anti-GBM glomerulonephritis Mass spectrometry Exostosin ½ |
| url | https://doi.org/10.1186/s12882-023-03268-1 |
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