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

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.

SUBMITTER: Imaizumi R

PROVIDER: S-EPMC5411619 | biostudies-literature | 2015 May

REPOSITORIES: biostudies-literature

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

Imaizumi Rie R Ishii Yasuo Y Miki Katsuyuki K Kudo Shinji S Murakami Toru T Koyama Ichiro I Nakajima Ichiro I Fuchinoue Shohei S Kawaguchi Yasushi Y Taniguchi Atsuo A Teraoka Satoshi S

CEN case reports 20140614 1

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 ...[more]

PMID: 28509262

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Cryopyrin-associated periodic syndrome: an update on diagnosis and treatment response.

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| S-EPMC4323990 | biostudies-literature

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