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Nephrotic syndrome induced by aortic regurgitation with Takayasu arteritis: an autopsy case with long-term clinical follow-up

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Abstract

Takayasu arteritis is a rare, chronic, and large-vessel vasculitis involving the aorta and its branches in a complex autoimmune reaction. Takayasu arteritis sometimes complicates aortic regurgitation and chronic kidney disease, but rarely accompanies nephrotic syndrome. We had a patient with Takayasu arteritis and concomitant aortic regurgitation. She had nephrotic syndrome that was refractory to immunosuppressive therapy but was promptly improved after surgical aortic valve replacement. In her kidney biopsy, glomeruli had mild mesangial proliferative changes without immune complex deposition. Her proteinuria remained negative until the recurrence of aortic regurgitation due to perivalvular leakage. Seventeen years after the surgery, she died suddenly. In her kidney autopsy, the arteriolar showed severe hyalinosis and the glomerulus showed mesangial proliferative changes with segmental mesangiolysis. Severe aortic regurgitation may have altered renal hemodynamics and caused glomerular lesions, resulting in nephrotic syndrome. We should be aware of the rare but critical comorbidity of nephrotic syndrome in patients with Takayasu arteritis and concomitant aortic regurgitation.

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Authors and Affiliations

  1. The Second Department of Internal Medicine, University of Toyama, 2630 Sugitani, Toyama, 930-0194, Japan

    Kota Kakeshita, Teruhiko Imamura, Sayaka Murai, Hayato Fujioka, Hidenori Yamazaki, Tsutomu Koike & Koichiro Kinugawa

  2. Department of Diagnostic Pathology, University of Toyama, Toyama, Japan

    Akira Noguchi

Authors
  1. Kota Kakeshita
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  2. Teruhiko Imamura
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  3. Akira Noguchi
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  4. Sayaka Murai
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  5. Hayato Fujioka
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  6. Hidenori Yamazaki
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  7. Tsutomu Koike
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  8. Koichiro Kinugawa
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Correspondence to Teruhiko Imamura.

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All procedures performed in this case were in accordance with the ethical standards of the 1964 Helsinki Declaration.

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Kakeshita, K., Imamura, T., Noguchi, A. et al. Nephrotic syndrome induced by aortic regurgitation with Takayasu arteritis: an autopsy case with long-term clinical follow-up. CEN Case Rep (2023). https://doi.org/10.1007/s13730-023-00819-1

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  • DOI: https://doi.org/10.1007/s13730-023-00819-1

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