PMID- 37488532 OWN - NLM STAT- MEDLINE DCOM- 20230809 LR - 20230809 IS - 1471-2369 (Electronic) IS - 1471-2369 (Linking) VI - 24 IP - 1 DP - 2023 Jul 24 TI - 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. PG - 218 LID - 10.1186/s12882-023-03268-1 [doi] LID - 218 AB - 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. CI - (c) 2023. The Author(s). FAU - Yamazaki, Takuya AU - Yamazaki T AD - Department of Nephrology, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-Ku, SagamiharaKanagawa, 252-0374, Japan. FAU - Takahashi, Haruka AU - Takahashi H AD - Department of Nephrology, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-Ku, SagamiharaKanagawa, 252-0374, Japan. AD - Omigawahimawari Clinic, Chiba, Japan. FAU - Takeuchi, Kazuhiro AU - Takeuchi K AD - Department of Nephrology, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-Ku, SagamiharaKanagawa, 252-0374, Japan. takekazu04044@gmail.com. AD - Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan. takekazu04044@gmail.com. FAU - Sakamoto, Emi AU - Sakamoto E AD - Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan. FAU - Tominaga, Kenta AU - Tominaga K AD - Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan. FAU - Sakurabayashi, Syun AU - Sakurabayashi S AD - Department of Nephrology, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-Ku, SagamiharaKanagawa, 252-0374, Japan. FAU - Abe, Tetsuya AU - Abe T AD - Department of Nephrology, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-Ku, SagamiharaKanagawa, 252-0374, Japan. FAU - Sano, Takashi AU - Sano T AD - Aihara Hospital, Kanagawa, Japan. FAU - Wada, Yukihiro AU - Wada Y AD - Department of Nephrology, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-Ku, SagamiharaKanagawa, 252-0374, Japan. FAU - Kuwahara, Naomi AU - Kuwahara N AD - Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan. FAU - Shimizu, Akira AU - Shimizu A AD - Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan. FAU - Takeuchi, Yasuo AU - Takeuchi Y AD - Department of Nephrology, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-Ku, SagamiharaKanagawa, 252-0374, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20230724 PL - England TA - BMC Nephrol JT - BMC nephrology JID - 100967793 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 0 (antiglomerular basement membrane antibody) RN - 0 (Immunoglobulin G) RN - EC 2.4.1.- (N-Acetylglucosaminyltransferases) RN - EC 2.4.1.224 (exostosin-1) RN - EC 2.4.1.224 (exostosin-2) SB - IM MH - Aged MH - Humans MH - Male MH - *Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications MH - Antibodies, Antineutrophil Cytoplasmic MH - *Glomerulonephritis/pathology MH - *Glomerulonephritis, Membranoproliferative/complications MH - *Glomerulonephritis, Membranous/complications/diagnosis MH - Immunoglobulin G MH - *Lupus Erythematosus, Systemic/complications MH - *Lupus Nephritis/complications/diagnosis MH - Mass Spectrometry MH - N-Acetylglucosaminyltransferases PMC - PMC10364369 OTO - NOTNLM OT - ANCA-associated vasculitis OT - Anti-GBM glomerulonephritis OT - Exostosin (1/2) OT - Lupus nephritis OT - Mass spectrometry COIS- The authors declare no competing interests. EDAT- 2023/07/25 01:09 MHDA- 2023/07/28 06:42 PMCR- 2023/07/24 CRDT- 2023/07/24 23:41 PHST- 2023/04/01 00:00 [received] PHST- 2023/07/13 00:00 [accepted] PHST- 2023/07/28 06:42 [medline] PHST- 2023/07/25 01:09 [pubmed] PHST- 2023/07/24 23:41 [entrez] PHST- 2023/07/24 00:00 [pmc-release] AID - 10.1186/s12882-023-03268-1 [pii] AID - 3268 [pii] AID - 10.1186/s12882-023-03268-1 [doi] PST - epublish SO - BMC Nephrol. 2023 Jul 24;24(1):218. doi: 10.1186/s12882-023-03268-1.