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A case of cryopyrin-associated periodic syndrome with kidney transplant failure

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Abstract

The cryopyrin-associated periodic syndrome (CAPS) is an autosomal dominant autoinflammatory disease characterized by fever, skin rash, and joint involvement with acute inflammatory response. The genetic defect involves the NLRP3 gene that encodes cryopyrin and leads to an abnormal production of interleukin-1 (IL-1). Therefore, anti-IL-1 treatment represents an effective therapy. One of the most severe manifestations of the disease is secondary amyloidosis that causes renal failure. We present a patient with CAPS who underwent renal transplantation for renal insufficiency caused by amyloidosis. The function of the transplanted kidney deteriorated because of the late administration of IL-1 receptor antagonist, anakinra. This case may indicate the importance of early initiation of anti-IL-1 treatment in CAPS patients who have undergone kidney transplantation.

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Authors and Affiliations

  1. Department of Surgery, Kidney Center, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan

    Rie Imaizumi, Yasuo Ishii, Katsuyuki Miki, Shinji Kudo, Toru Murakami, Ichiro Koyama, Ichiro Nakajima, Shohei Fuchinoue & Satoshi Teraoka

  2. Institute of Rheumatology, Tokyo Women’s Medical University, 10-22 Kawada-cho, Shinjuku-ku, Tokyo, 162-0054, Japan

    Yasushi Kawaguchi & Atsuo Taniguchi

Authors
  1. Rie Imaizumi
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  2. Yasuo Ishii
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  3. Katsuyuki Miki
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  4. Shinji Kudo
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  5. Toru Murakami
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  6. Ichiro Koyama
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  7. Ichiro Nakajima
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  8. Shohei Fuchinoue
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  9. Yasushi Kawaguchi
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  10. Atsuo Taniguchi
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  11. Satoshi Teraoka
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Corresponding author

Correspondence to Atsuo Taniguchi.

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Imaizumi, R., Ishii, Y., Miki, K. et al. A case of cryopyrin-associated periodic syndrome with kidney transplant failure. CEN Case Rep 4, 1–5 (2015). https://doi.org/10.1007/s13730-014-0129-y

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  • DOI: https://doi.org/10.1007/s13730-014-0129-y

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