1. A case of cryopyrin-associated periodic syndrome with kidney transplant failure
- Author
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Yasuo Ishii, Katsuyuki Miki, Toru Murakami, Yasushi Kawaguchi, Ichiro Nakajima, Satoshi Teraoka, Atsuo Taniguchi, Ichiro Koyama, Shinji Kudo, Shohei Fuchinoue, and Rie Imaizumi
- Subjects
Nephrology ,Anakinra ,medicine.medical_specialty ,integumentary system ,business.industry ,Amyloidosis ,Cryopyrin-associated periodic syndrome ,Case Report ,General Medicine ,Disease ,medicine.disease ,Rash ,Gastroenterology ,Transplantation ,Internal medicine ,Immunology ,medicine ,medicine.symptom ,business ,Kidney transplantation ,medicine.drug - 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.
- Published
- 2014
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