CEN Case Reports

, Volume 4, Issue 1, pp 1–5 | Cite as

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

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

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

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

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Copyright information

© Japanese Society of Nephrology 2014

Authors and Affiliations

  • Rie Imaizumi
    • 1
  • Yasuo Ishii
    • 1
  • Katsuyuki Miki
    • 1
  • Shinji Kudo
    • 1
  • Toru Murakami
    • 1
  • Ichiro Koyama
    • 1
  • Ichiro Nakajima
    • 1
  • Shohei Fuchinoue
    • 1
  • Yasushi Kawaguchi
    • 2
  • Atsuo Taniguchi
    • 2
  • Satoshi Teraoka
    • 1
  1. 1.Department of Surgery, Kidney CenterTokyo Women’s Medical UniversityTokyoJapan
  2. 2.Institute of RheumatologyTokyo Women’s Medical UniversityTokyoJapan

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