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

被引：0
作者：
Rie Imaizumi
Yasuo Ishii
Katsuyuki Miki
Shinji Kudo
Toru Murakami
Ichiro Koyama
Ichiro Nakajima
Shohei Fuchinoue
Yasushi Kawaguchi
Atsuo Taniguchi
Satoshi Teraoka
机构：
[1] Tokyo Women’s Medical University,Department of Surgery, Kidney Center
[2] Tokyo Women’s Medical University,Institute of Rheumatology
来源：
CEN Case Reports | 2015年 / 4卷 / 1期
关键词：
Cryopyrin-associated periodic syndrome; Autoinflammatory disease; Interleukin-1; Amyloidosis; Kidney transplantation; Anakinra;
D O I：
10.1007/s13730-014-0129-y
中图分类号：
学科分类号：
摘要：
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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页码：1 / 5
页数：4
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