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

CEN Case Reports
Rie ImaizumiSatoshi Teraoka

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.

References

Aug 12, 2005·Current Opinion in Rheumatology·Silvia Stojanov, Daniel L Kastner
Aug 11, 2006·The New England Journal of Medicine·Raphaela Goldbach-ManskyDaniel L Kastner
Mar 6, 2007·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Brian D ThorntonBurl R Don
Nov 27, 2008·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Christian MoserJosef Kovarik
Jun 6, 2009·The New England Journal of Medicine·Helen J LachmannUNKNOWN Canakinumab in CAPS Study Group
Jul 9, 2010·Joint, Bone, Spine : Revue Du Rhumatisme·Tassadit Aït-AbdesselamOlivier Vittecoq
Mar 2, 2011·Arthritis and Rheumatism·Jasmin B Kuemmerle-DeschnerSusanne M Benseler
May 4, 2011·Current Rheumatology Reports·Raphaela Goldbach-Mansky
Nov 22, 2012·American Journal of Transplantation : Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons·J H PinneyJ D Gillmore
Jan 3, 2013·Arthritis and Rheumatism·Thirusha LaneHelen J Lachmann
May 31, 2013·Arthritis Research & Therapy·Jasmin B Kuemmerle-DeschnerSusanne M Benseler

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Citations

Apr 14, 2020·The Journal of Dermatological Treatment·Kyle TegtmeyerPeter A Lio

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