Tubulointerstitial Nephritis Cases With IgM-Positive Plasma Cells

Matsuoka-Uchiyama, Natsumi; Tsuji, Kenji; Fukushima, Kazuhiko; Kitamura, Shinji; Uchida, Haruhito A.; Sugiyama, Hitoshi; Takahashi, Naoki; Iwano, Masayuki; Wada, Jun

doi:10.1016/j.ekir.2020.06.010

Cited by 5 publications

(5 citation statements)

References 8 publications

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“…Although its pathophysiological mechanisms are still largely unknown, the major clinical features of IgMPC-TIN are high s-IgM levels; high prevalence (> 80%) of d-RTA, Fanconi syndrome and positivity for AMA; and complication of PBC (46%) or Sjӧgren syndrome (31%) [ 2 ]. In some cases of IgMPC-TIN, liver biopsies have shown that IgM-PCs infiltrate in the portal tract [ 6 , 8 ], which suggests that IgM-positive plasma cells themselves may be responsible for major syndromes such as TIN, dRTA, Fanconi syndrome, and PBC.…”

Section: Discussionmentioning

confidence: 99%

“…Although initial glucocorticoid therapy is reported to be effective [ 2 ], there is no clear definition of relapse or consensus around treating a recurrence. To the best of our knowledge, there are only four case reports of relapse; in three of the four cases, s-IgM and u-β 2 MG trends during the glucocorticoid taper were described in detail [ 6 – 8 ]. The clinical features of six patients with relapse, including our three patients, are described below.…”

Section: Discussionmentioning

confidence: 99%

“…Matsuoka-Uchiyama et al reported that proteinuria, glycosuria, u-β 2 MG, and s-IgM are useful markers for detecting relapse [ 6 ]. In all three of our patients, s-IgM increased prior to re-elevation of u-β 2 MG, urine protein levels, and renal dysfunction.…”

Section: Discussionmentioning

confidence: 99%

“…Pathologically, IgMPC-TIN is characterized by accumulation of IgM-positive plasma cells (identified using IgM/CD138 immunohistochemistry) within the interstitium, whereas conventional TIN is characterized by infiltration with IgG-positive cells. In most cases of IgMPC-TIN, intermediate-dose glucocorticoid therapy is highly effective, but there are a number of reports of patients relapsing during glucocorticoid tapering [ 6 – 8 ]. There is no clear definition of relapse or consensus of when treatment should be intensified.…”

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: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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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“…A few cases of recurrent IgMPC-TIN have been reported. Matsuoka-Uchiyama et al reported two cases of relapse after rapid tapering of glucocorticoids (36). Mizoguchi et al reported a case of recurrent IgMPC-TIN associated with PBC after steroid tapering that improved following the administration of 500 mg of methylprednisolone per day for 3 consecutive days, followed by 10 mg of PSL daily (37).…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2021

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Tubulointerstitial nephritis (TIN) with IgM-positive plasma cells (IgMPC-TIN) is an autoimmune kidney disease characterized by IgM/CD138-double-positive plasma cell infiltration in the tubulointerstitium. A 50year-old man developed IgMPC-TIN and presented with crystalline inclusions in the rough endoplasmic reticulum. Intracellular crystal formation is a rare finding in paraprotein-related kidney diseases, but this case showed no pathogenic monoclonal immunoglobulin. Prednisolone (PSL, 30 mg) improved the TIN, but PSL tapering resulted in the recurrence of TIN. Combination therapy with 15 mg PSL and 150 mg mizoribine ultimately stabilized TIN. This case offers original evidence concerning the pathophysiology and treatment strategy of IgMPC-TIN.

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Tubulointerstitial nephritis with IgM-positive plasma cells complicated by liver failure

Kudo,

Nakazawa,

Nishio

et al. 2024

CEN Case Rep

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Tubulointerstitial Nephritis Cases With IgM-Positive Plasma Cells

Cited by 5 publications

References 8 publications

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

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

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

Tubulointerstitial nephritis with IgM-positive plasma cells complicated by liver failure

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