A CASE OF TUBULOINTERSTITIAL NEPHRITIS WITH IGM-POSITIVE PLASMA CELLS REQUIRING TEMPORARY HEMODIALYSIS

 

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https://storage.unitedwebnetwork.com/files/1099/2032f17cd306a330ce0d6fec84279307.pdf
A CASE OF TUBULOINTERSTITIAL NEPHRITIS WITH IGM-POSITIVE PLASMA CELLS REQUIRING TEMPORARY HEMODIALYSIS

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Hisashi
Furukawa
Hisashi Furukawa furukawa.hisashi@1972.saitama-med.ac.jp Saitama Medical University Department of Nephrology Saitama Japan *
Tsutomu Inoue t_inoue@saitama-med.ac.jp Saitama Medical University Department of Nephrology Saitama Japan -
Haruko Oba ooba-haruko@saitama-med.ac.jp Saitama Medical University Department of Nephrology Saitama Japan -
Tatsuo Kondo tatkondo@saitama-med.ac.jp Saitama Medical University Department of Nephrology Saitama Japan -
Daichi Fukaya dora2222@saitama-med.ac.jp Saitama Medical University Department of Nephrology Saitama Japan -
Koji Tomori tomori@saitama-med.ac.jp Saitama Medical University Department of Nephrology Saitama Japan -
Yusuke Watanabe yusuke_w@saitama-med.ac.jp Saitama Medical University Department of Nephrology Saitama Japan -
Hirokazu Okada hirookda@saitama-med.ac.jp Saitama Medical University Department of Nephrology Saitama Japan -
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Tubulointerstitial nephritis with IgM-positive plasma cells (IgMPC-TIN) was first identified in Japan in 2017 and is recognized as a cause of acute kidney injury (AKI). Although glucocorticoid therapy is generally effective, a minority of cases have required dialysis. We present a case of IgMPC-TIN necessitating temporary hemodialysis, followed by successful withdrawal from dialysis after initiation of glucocorticoid therapy.

Case Presentation; A 63-year-old Japanese woman presented with fatigue. Ten years earlier, she had been diagnosed with drug-induced acute tubulointerstitial nephritis, which resolved following discontinuation of the suspected agent. Her renal function remained stable thereafter, with a serum creatinine level of 1.1 mg/dL. Five days prior to admission, she developed fatigue and was found to have severe renal dysfunction (serum creatinine 7.12 mg/dL), prompting hospitalization. Hemodialysis was initiated two days after admission. Laboratory findings revealed marked elevation of urinary β2-microglobulin, metabolic acidosis (plasma pH 7.14; bicarbonate 12.7 mmol/L), and urinary losses of uric acid, phosphate, glucose, and amino acids, consistent with Fanconi syndrome. Serum IgM was markedly elevated at 2650 mg/dL. Renal biopsy revealed seven glomeruli, two of which were globally sclerosed. No spikes or double contours were observed in the glomerular basement membrane. The tubulointerstitium showed diffuse lymphocytic infiltration, moderate tubular atrophy, and interstitial fibrosis. Immunohistochemistry demonstrated infiltration of CD3-, CD138-, and IgM-positive cells. Double staining for IgM and CD138 revealed that 42% of CD138-positive cells co-expressed IgM. Based on these findings, a diagnosis of IgMPC-TIN was established.

Clinical Course; Prednisolone (30 mg/day) was initiated on hospital day 23. Renal function gradually improved, allowing discontinuation of hemodialysis 72 days after admission.

Serum IgM levels have been proposed as a marker of disease activity in IgMPC-TIN. In comparison with previously reported cases, this patient exhibited the highest serum IgM level, suggesting a particularly active disease state. The severity of renal dysfunction requiring dialysis may reflect this heightened activity. Importantly, renal function improved following glucocorticoid therapy, underscoring the potential reversibility of even severe AKI in IgMPC-TIN. Clinicians should consider IgMPC-TIN in patients presenting with AKI and elevated serum IgM, as timely diagnosis and intervention may significantly improve renal outcomes.

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