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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. 2014;4(1):1-5doi: 10.1007/s13730-014-0129-y.
Imaizumi, R., Ishii, Y., Miki, K., Kudo, S., Murakami, T., Koyama, I., Nakajima, I., Fuchinoue, S., Kawaguchi, Y., Taniguchi, A., & Teraoka, S. (2015). A case of cryopyrin-associated periodic syndrome with kidney transplant failure. CEN case reports, 4(1), 1-5. https://doi.org/10.1007/s13730-014-0129-y
Imaizumi, Rie, et al. "A case of cryopyrin-associated periodic syndrome with kidney transplant failure." CEN case reports vol. 4,1 (2015): 1-5. doi: https://doi.org/10.1007/s13730-014-0129-y
Imaizumi R, Ishii Y, Miki K, Kudo S, Murakami T, Koyama I, Nakajima I, Fuchinoue S, Kawaguchi Y, Taniguchi A, Teraoka S. A case of cryopyrin-associated periodic syndrome with kidney transplant failure. CEN Case Rep. 2015 May;4(1):1-5. doi: 10.1007/s13730-014-0129-y. Epub 2014 Jun 14. PMID: 28509262; PMCID: PMC5411619.
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CEN Case Rep. 2015 May;4(1):1-5. doi: 10.1007/s13730-014-0129-y. Epub 2014 Jun 14.

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

CEN case reports

Rie Imaizumi, Yasuo Ishii, Katsuyuki Miki, Shinji Kudo, Toru Murakami, Ichiro Koyama, Ichiro Nakajima, Shohei Fuchinoue, Yasushi Kawaguchi, Atsuo Taniguchi, Satoshi Teraoka

Affiliations

  1. Department of Surgery, Kidney Center, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.
  2. Institute of Rheumatology, Tokyo Women's Medical University, 10-22 Kawada-cho, Shinjuku-ku, Tokyo, 162-0054, Japan.
  3. Institute of Rheumatology, Tokyo Women's Medical University, 10-22 Kawada-cho, Shinjuku-ku, Tokyo, 162-0054, Japan. [email protected].

PMID: 28509262 PMCID: PMC5411619 DOI: 10.1007/s13730-014-0129-y

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.

Keywords: Amyloidosis; Anakinra; Autoinflammatory disease; Cryopyrin-associated periodic syndrome; Interleukin-1; Kidney transplantation

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