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

• 发表时间: May 2015
• 来源期刊: CEN Case Rep
• DOI: 10.1007/s13730-014-0129-y
• PMID: 28509262
• 文章类型: Journal Article
• 作者列表: Imaizumi R,Ishii Y,Miki K,Kudo S,Murakami T,Koyama I,Nakajima I,Fuchinoue S,Kawaguchi Y,Taniguchi A,Teraoka S

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

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.