Glucocorticoid-dependent Tubulointerstitial Nephritis with IgM-positive Plasma Cells Presenting with Intracellular Crystalline Inclusions within the Rough Endoplasmic Reticulum

Minato, M.; Murakami, Taichi; Takahashi, Naoki; Ono, Hiroyuki; Nishimura, Kenji; Tamaki, Masanori; Nagai, Kojiro; Abe, Hideharu; Iwano, Masayuki; Joh, Kensuke; Doi, Toshio

doi:10.2169/internalmedicine.7118-21

Cited by 1 publication

(2 citation statements)

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“…One of our patients (case 3) is overeating and becoming obese as a side effect of PSL treatment; we are considering reducing the PSL dose and adding immunosuppressants. Minato et al reported a good response after treatment with add-on cyclosporine A or mizoribine [ 7 ]. In cases of long-term glucocorticoid use, the addition of immunosuppressants such as cyclosporine A or mizoribine may be considered, although further data to support this approach are needed.…”

Section: Discussionmentioning

confidence: 99%

“…There is no clear definition of relapse or consensus of when treatment should be intensified. Minato et al reported that cyclosporine A or mizoribine in combination with prednisolone (PSL) was effective in controlling the disease in the case of relapse [ 7 ], but the efficacy and method of administration of immunosuppressants in relapse cases have not been established.…”

Section: Introductionmentioning

confidence: 99%

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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

et al. 2023

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%