DIAGNOSIS AND TREATMENT OF PATIENTS WITH CRYOPYRIN-ASSOCIATED PERIODIC SYNDROME
https://doi.org/10.15690/vsp.v14i2.1301
Abstract
Cryopyrin-Associated Periodic Syndrome (CAPS) refers to a group of autoinflammatory diseases and is caused by autosomal dominant mutations of the NLRP3 gene coding. Relapsing fever, rash, laboratory changes and inflammatory lesions of various organs are typical manifestations of CAPS. Inhibitors of interleukin 1, including canakinumab, are modern means of pathogenetic therapy of patients with CAPS. The article presents the treatment results in 5 pediatric patients with CAPS. Application of canakinumab led to the relief of all major symptoms (fever, inflammatory changes in the blood lymphoproliferation, signs of organ damage), and no relevant side effects was detected.
About the Authors
A. L. Kozlova
D. Rogachev Federal Research and Clinical Centre of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation
Russian Federation
Russian Federation
B. S. Pershin
D. Rogachev Federal Research and Clinical Centre of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation
Russian Federation
Russian Federation
T. V. Varlamova
D. Rogachev Federal Research and Clinical Centre of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation
Russian Federation
Russian Federation
A. Yu. Shcherbina
D. Rogachev Federal Research and Clinical Centre of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation
Russian Federation
Russian Federation
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Review
For citations:
Kozlova A.L., Pershin B.S., Varlamova T.V., Shcherbina A.Yu. DIAGNOSIS AND TREATMENT OF PATIENTS WITH CRYOPYRIN-ASSOCIATED PERIODIC SYNDROME. Current Pediatrics. 2015;14(2):292-297. (In Russ.) https://doi.org/10.15690/vsp.v14i2.1301
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