Membranous nephropathy preceding Bing-Neel syndrome: successful treatment with tirabrutinib

Watanabe-Kusunoki, Kanako; Nakazawa, Daigo; Ohigashi, Hiroyuki; Tsuji, Takahiro; Atsumi, Tatsuya

Membranous nephropathy preceding Bing-Neel syndrome: successful treatment with tirabrutinib

Watanabe-Kusunoki, Kanako; Nakazawa, Daigo; Ohigashi, Hiroyuki et al.

Journal of nephrology, 2025, 38(9), 2991-2996

Permalink : https://hdl.handle.net/2115/98145

このアイテムのアクセス数:23件（2026-10-17 12:20 集計）

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論文情報

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アクセス権	open access
URI	https://hdl.handle.net/2115/98145
タイトル	Membranous nephropathy preceding Bing-Neel syndrome: successful treatment with tirabrutinib
著者	Watanabe-Kusunoki, Kanako ; Nakazawa, Daigo ; Ohigashi, Hiroyuki ; Tsuji, Takahiro ; Atsumi, Tatsuya
e-Rad_Researcher 21000134 著者名 Watanabe-Kusunoki, Kanako
e-Rad_Researcher 60724135 著者名 Nakazawa, Daigo
e-Rad_Researcher 20845284 著者名 Ohigashi, Hiroyuki
著者名 Tsuji, Takahiro
e-Rad_Researcher 20301905 著者名 Atsumi, Tatsuya
言語	英語
キーワード	Bing-Neel syndrome
	Membranous nephropathy
	Tirabrutinib
	Waldenström macroglobulinemia
発行日	2025-09-26
出版者	Springer
収録物名	Journal of nephrology
巻(号)	38(9)
ページ	2991-2996
抄録	We report a unique case of membranous nephropathy and tubulointerstitial nephritis preceding neurological manifestations of Bing-Neel syndrome, a rare variant of Waldenstr & ouml;m macroglobulinemia. A 46-year-old man presented with nephrotic syndrome, and kidney biopsy revealed membranous nephropathy with phospholipase A2 receptor positivity and IgG4 predominance. Despite initial partial remission with corticosteroids and cyclosporine, neurological symptoms emerged alongside nephrotic syndrome relapse two years after onset. Diagnostic workup revealed serum monoclonal IgM kappa, abnormal lymphoplasmacytic cells in bone marrow and cerebrospinal fluid, and MYD88 L265P mutation in the latter, confirming Bing-Neel syndrome. Immunostaining of the initial kidney biopsy demonstrated IgM-kappa-positive lymphoplasmacytic infiltration consistent with the monoclonal protein. After bendamustine-rituximab therapy showed partial effectiveness, tirabrutinib, a Bruton tyrosine kinase inhibitor, achieved complete remission of both renal and neurological manifestations with sustained response over three years. Immunofluorescence staining revealed increased nuclear factor (NF)-kappa B and STAT3 expression in infiltrating immune cells and elevated NF-kappa B in surrounding tubular epithelial cells, suggesting activation of these pathways downstream of the MYD88 mutation in kidney pathology. Our findings suggest a potential contribution of Bruton's tyrosine kinase signaling to both kidney and neurological pathology in Bing-Neel syndrome, with tirabrutinib achieving clinical improvement in both organ manifestations.
資源タイプ	学術雑誌論文
出版タイプ	Accepted Manuscript
権利情報	This version of the article has been accepted for publication, after peer review (when applicable) and is subject to Springer Nature’s AM terms of use, but is not the Version of Record and does not reflect post-acceptance improvements, or any corrections. The Version of Record is available online at: http://dx.doi.org/10.1007/s40620-025-02401-7
PISSN	1121-8428
EISSN	1724-6059
関連情報	URI https://rdcu.be/eIoYe
関連情報 (isVersionOf)	出版社版DOI https://doi.org/10.1007/s40620-025-02401-7