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

被引：0

作者：

Imaizumi, Rie ^{[1
]}

Ishii, Yasuo ^{[1
]}

Miki, Katsuyuki ^{[1
]}

Kudo, Shinji ^{[1
]}

Murakami, Toru ^{[1
]}

Koyama, Ichiro ^{[1
]}

Nakajima, Ichiro ^{[1
]}

Fuchinoue, Shohei ^{[1
]}

Kawaguchi, Yasushi ^{[2
]}

Taniguchi, Atsuo ^{[2
]}

Teraoka, Satoshi ^{[1
]}

机构：

[1] Tokyo Womens Med Univ, Dept Surg, Kidney Ctr, Shinjuku Ku, Tokyo 1628666, Japan

[2] Tokyo Womens Med Univ, Inst Rheumatol, Shinjuku Ku, 10-22 Kawada Cho, Tokyo 1620054, Japan

来源：

CEN CASE REPORTS | 2015年 / 4卷 / 01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：

R5 [内科学]; R69 [泌尿科学（泌尿生殖系疾病）];

学科分类号：

1002 ; 100201 ;

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.

引用

页码：1 / 5

页数：5

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