Urinary β2-microglobulin as a Potential Biomarker for Early Treatment Selection: A Patient with Severe IgG4-related Tubulointerstitial Nephritis

Nozomi Taniguchi; Masato Sakai; Takahiro Masuda; Chihiro Otsuki; Maki Asakura-Kinoshita; Kyohei Misawa; Kazuho Oe; Kazutoshi Ono; Natsuko Wakabayashi; Kentaro Oka; Erika Hishida; Yuko Ono; Tetsu Akimoto; Daisuke Nagata

doi:10.2169/internalmedicine.5407-25

Nozomi Taniguchi, Masato Sakai, Takahiro Masuda, Chihiro Otsuki, Maki Asakura-Kinoshita, Kyohei Misawa, Kazuho Oe, Kazutoshi Ono, Natsuko Wakabayashi, Kentaro Oka, Erika Hishida, Yuko Ono, Tetsu Akimoto, Daisuke Nagata

Author information

Nozomi Taniguchi
Division of Nephrology, Department of Medicine, Jichi Medical University, Japan
Masato Sakai
Division of Nephrology, Department of Medicine, Jichi Medical University, Japan
Takahiro Masuda
Division of Nephrology, Department of Medicine, Jichi Medical University, Japan
Chihiro Otsuki
Division of Nephrology, Department of Medicine, Jichi Medical University, Japan
Maki Asakura-Kinoshita
Division of Nephrology, Department of Medicine, Jichi Medical University, Japan
Kyohei Misawa
Division of Nephrology, Department of Medicine, Jichi Medical University, Japan
Kazuho Oe
Division of Nephrology, Department of Medicine, Jichi Medical University, Japan
Kazutoshi Ono
Division of Nephrology, Department of Medicine, Jichi Medical University, Japan
Natsuko Wakabayashi
Division of Nephrology, Department of Medicine, Jichi Medical University, Japan
Kentaro Oka
Division of Nephrology, Department of Medicine, Jichi Medical University, Japan
Erika Hishida
Division of Nephrology, Department of Medicine, Jichi Medical University, Japan
Yuko Ono
Department of Diagnostic Pathology, Dokkyo Medical University, Japan
Department of Nephrology and Hypertension, Saitama Medical Center, Saitama Medical University, Japan
Tetsu Akimoto
Division of Nephrology, Department of Medicine, Jichi Medical University, Japan
Daisuke Nagata
Division of Nephrology, Department of Medicine, Jichi Medical University, Japan

Keywords: IgG4-related kidney disease, IgG4-related tubulointerstitial nephritis, urinary β2-microglobulin, tubular marker, steroid pulse, biomarker

JOURNAL OPEN ACCESS Advance online publication

Article ID: 5407-25

DOI https://doi.org/10.2169/internalmedicine.5407-25

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Abstract

This case report describes an 80-year-old man with severe immunoglobulin G4-related tubulointerstitial nephritis (IgG4-TIN), characterized by storiform fibrosis with diffuse lymphocytic and plasma cell infiltration observed on a renal biopsy. Steroid pulse therapy administered immediately after confirming a remarkable increase in urinary β2-microglobulin (100,948 μg/L) along with no evidence of malignancy significantly improved the renal function and reduced the urinary β2-microglobulin levels. This study highlights the potential utility of urinary β2-microglobulin as a biomarker for early treatment selection in severe IgG4-TIN and emphasizes the need for timely intervention to prevent irreversible kidney damage.

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