A report of three cases of patients with tubulointerstitial nephritis with IgM-positive plasma cells, treatment, and serum-IgM as a sensitive marker for relapse
Abstract Background Tubulointerstitial nephritis with IgM-positive plasma cells (IgMPC-TIN) is a newer disease about which there are many unclear points. Glucocorticoid therapy is effective in many cases of IgMPC-TIN; however, relapse during glucocorticoid tapering has been reported. Relapse and its...
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| Format: | Article |
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BMC
2023-07-01
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| Series: | BMC Nephrology |
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| Online Access: | https://doi.org/10.1186/s12882-023-03253-8 |
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| author | Ryota Akagi Akira Ishii Keiichi Kaneko Naoya Kondo Hideki Yokoi Takeshi Matsubara Sachiko Minamiguchi Yoshihiko Kanno Motoko Yanagita |
| author_facet | Ryota Akagi Akira Ishii Keiichi Kaneko Naoya Kondo Hideki Yokoi Takeshi Matsubara Sachiko Minamiguchi Yoshihiko Kanno Motoko Yanagita |
| author_sort | Ryota Akagi |
| collection | DOAJ |
| description | Abstract Background Tubulointerstitial nephritis with IgM-positive plasma cells (IgMPC-TIN) is a newer disease about which there are many unclear points. Glucocorticoid therapy is effective in many cases of IgMPC-TIN; however, relapse during glucocorticoid tapering has been reported. Relapse and its treatment are poorly defined. Case Presentation Case 1 was a 61-year-old man with renal dysfunction and proteinuria. Tubulointerstitial nephritis and IgM-positive plasma cells were observed in a renal biopsy. He was diagnosed with IgMPC-TIN accompanied by Fanconi syndrome and distal renal tubular acidosis (d-RTA). Prednisolone (PSL; 30 mg daily, 0.45 mg/kg/day) treatment was highly effective, and PSL was gradually tapered and discontinued after 1 year. However, 1 month after PSL discontinuation, therapeutic markers were elevated. Therefore, PSL (10 mg daily, 0.15 mg/kg/day) was administered, and the markers indicated improvement. Case 2 was a 43-year-old woman referred for renal dysfunction and proteinuria. Laboratory data revealed that she had primary biliary cholangitis (PBC), d-RTA, and Fanconi syndrome. A renal biopsy showed accumulation of IgM-positive plasma cells in the tubulointerstitium without any glomerular changes. A diagnosis of IgMPC-TIN was made and the patient was started on PSL (35 mg daily, 0.6 mg/kg/day). Therapeutic markers decreased immediately and PSL was discontinued after 1 year. Three months later, the proteinuria and Fanconi syndrome worsened. PSL treatment was restarted (20 mg daily, 0.35 mg/kg/day) and markers indicated improvement. Case 3 was a 45-year-old woman with renal dysfunction and proteinuria. Tubulointerstitial nephritis and IgM-positive plasma cells were observed in a renal biopsy. The patient had PBC, Sjögren syndrome, d-RTA, and Fanconi syndrome, and the diagnosis of IgMPC-TIN was made. The patient was started on PSL (30 mg daily, 0.4 mg/kg/day) and disease markers decreased immediately. However, when PSL was tapered to 15 mg daily (0.2 mg/kg/day), the patient’s serum IgM levels increased; therefore, we maintained the PSL at 15 mg daily (0.2 mg/kg/day). Conclusion We report three cases of relapsed IgMPC-TIN associated with reduction or discontinuation of glucocorticoid therapy. In these cases, elevation of serum IgM preceded that of other markers such as urinary β2-microglobulin, proteinuria, and glycosuria. We recommend monitoring serum IgM levels while tapering glucocorticoids; a maintenance dose of glucocorticoid should be considered if relapse is suspected or anticipated. |
| first_indexed | 2024-03-13T00:44:24Z |
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| language | English |
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| publishDate | 2023-07-01 |
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| spelling | doaj.art-49d6c564e6e64ce28badca183768bf822023-07-09T11:08:40ZengBMCBMC Nephrology1471-23692023-07-012411710.1186/s12882-023-03253-8A report of three cases of patients with tubulointerstitial nephritis with IgM-positive plasma cells, treatment, and serum-IgM as a sensitive marker for relapseRyota Akagi0Akira Ishii1Keiichi Kaneko2Naoya Kondo3Hideki Yokoi4Takeshi Matsubara5Sachiko Minamiguchi6Yoshihiko Kanno7Motoko Yanagita8Department of Nephrology, Graduate School of Medicine, Kyoto UniversityDepartment of Nephrology, Graduate School of Medicine, Kyoto UniversityDepartment of Nephrology, Graduate School of Medicine, Kyoto UniversityDepartment of Nephrology, Graduate School of Medicine, Kyoto UniversityDepartment of Nephrology, Graduate School of Medicine, Kyoto UniversityDepartment of Nephrology, Graduate School of Medicine, Kyoto UniversityDepartment of Diagnostic Pathology, Graduate School of Medicine, Kyoto UniversityDepartment of Nephrology, Tokyo Medical UniversityDepartment of Nephrology, Graduate School of Medicine, Kyoto UniversityAbstract Background Tubulointerstitial nephritis with IgM-positive plasma cells (IgMPC-TIN) is a newer disease about which there are many unclear points. Glucocorticoid therapy is effective in many cases of IgMPC-TIN; however, relapse during glucocorticoid tapering has been reported. Relapse and its treatment are poorly defined. Case Presentation Case 1 was a 61-year-old man with renal dysfunction and proteinuria. Tubulointerstitial nephritis and IgM-positive plasma cells were observed in a renal biopsy. He was diagnosed with IgMPC-TIN accompanied by Fanconi syndrome and distal renal tubular acidosis (d-RTA). Prednisolone (PSL; 30 mg daily, 0.45 mg/kg/day) treatment was highly effective, and PSL was gradually tapered and discontinued after 1 year. However, 1 month after PSL discontinuation, therapeutic markers were elevated. Therefore, PSL (10 mg daily, 0.15 mg/kg/day) was administered, and the markers indicated improvement. Case 2 was a 43-year-old woman referred for renal dysfunction and proteinuria. Laboratory data revealed that she had primary biliary cholangitis (PBC), d-RTA, and Fanconi syndrome. A renal biopsy showed accumulation of IgM-positive plasma cells in the tubulointerstitium without any glomerular changes. A diagnosis of IgMPC-TIN was made and the patient was started on PSL (35 mg daily, 0.6 mg/kg/day). Therapeutic markers decreased immediately and PSL was discontinued after 1 year. Three months later, the proteinuria and Fanconi syndrome worsened. PSL treatment was restarted (20 mg daily, 0.35 mg/kg/day) and markers indicated improvement. Case 3 was a 45-year-old woman with renal dysfunction and proteinuria. Tubulointerstitial nephritis and IgM-positive plasma cells were observed in a renal biopsy. The patient had PBC, Sjögren syndrome, d-RTA, and Fanconi syndrome, and the diagnosis of IgMPC-TIN was made. The patient was started on PSL (30 mg daily, 0.4 mg/kg/day) and disease markers decreased immediately. However, when PSL was tapered to 15 mg daily (0.2 mg/kg/day), the patient’s serum IgM levels increased; therefore, we maintained the PSL at 15 mg daily (0.2 mg/kg/day). Conclusion We report three cases of relapsed IgMPC-TIN associated with reduction or discontinuation of glucocorticoid therapy. In these cases, elevation of serum IgM preceded that of other markers such as urinary β2-microglobulin, proteinuria, and glycosuria. We recommend monitoring serum IgM levels while tapering glucocorticoids; a maintenance dose of glucocorticoid should be considered if relapse is suspected or anticipated.https://doi.org/10.1186/s12882-023-03253-8Tubulointerstitial nephritis with IgM-positive plasma cellsRelapseSerum IgMDistal renal tubular acidosis (d-RTA)Fanconi syndromeSjögren syndrome |
| spellingShingle | Ryota Akagi Akira Ishii Keiichi Kaneko Naoya Kondo Hideki Yokoi Takeshi Matsubara Sachiko Minamiguchi Yoshihiko Kanno Motoko Yanagita A report of three cases of patients with tubulointerstitial nephritis with IgM-positive plasma cells, treatment, and serum-IgM as a sensitive marker for relapse BMC Nephrology Tubulointerstitial nephritis with IgM-positive plasma cells Relapse Serum IgM Distal renal tubular acidosis (d-RTA) Fanconi syndrome Sjögren syndrome |
| title | A report of three cases of patients with tubulointerstitial nephritis with IgM-positive plasma cells, treatment, and serum-IgM as a sensitive marker for relapse |
| title_full | A report of three cases of patients with tubulointerstitial nephritis with IgM-positive plasma cells, treatment, and serum-IgM as a sensitive marker for relapse |
| title_fullStr | A report of three cases of patients with tubulointerstitial nephritis with IgM-positive plasma cells, treatment, and serum-IgM as a sensitive marker for relapse |
| title_full_unstemmed | A report of three cases of patients with tubulointerstitial nephritis with IgM-positive plasma cells, treatment, and serum-IgM as a sensitive marker for relapse |
| title_short | A report of three cases of patients with tubulointerstitial nephritis with IgM-positive plasma cells, treatment, and serum-IgM as a sensitive marker for relapse |
| title_sort | report of three cases of patients with tubulointerstitial nephritis with igm positive plasma cells treatment and serum igm as a sensitive marker for relapse |
| topic | Tubulointerstitial nephritis with IgM-positive plasma cells Relapse Serum IgM Distal renal tubular acidosis (d-RTA) Fanconi syndrome Sjögren syndrome |
| url | https://doi.org/10.1186/s12882-023-03253-8 |
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