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
References
1. Kozlova A.L., Mamzerova E.S., Novichkova G.A., Shcherbina A.Yu. Clinical manifestations and therapy-associated kriopirin periodic syndromes. Voprosy gematologii/onkologii i immunopatologii v pediatrii = Issues of hematology/oncology and immunopathology in pediatrics. 2014; 13: 42-48.
2. Fietta P. Autoinflammatory disease: the hereditary periodic fever syndromes. Acta Biol. Ateneo Parmense. 2004; 75: 92–99.
3. Barabanova O.V., Konopleva E.A., Prodeus A.P., Shcherbina A.Yu. Periodic Syndromes. Trudn. patsient = Difficult patient. 2007; 2:46–52.
4. Martinon F., Tschopp J. Inflammatory caspases and inflammasomes: master switches of inflammation. Cell Death Differ. 2007; 14:10–22.
5. Aksentijevich I, Putman D, Remmers EF et al. The clinical continuum of cryopyrinopathies: novel CIAS1 mutations in North American patients and a new cryopyrin model. Arthritis Rheum. 2007; 56:1273-85.
6. Saito M., Nishikomori R., Kambe N., Fujisawa A., Tanizaki H., Takeichi K., et al. Disease-associated CIAS1 mutations induce monocyte death, revealing low-level mosaicism in mutationnegative cryopyrin-associated periodic syndrome patients. Blood. 2008; 111-2132-41.
7. Raphaela Goldbach-Mansky Current status of understanding the pathogenesis and management of patients wqith NOMID/CINCA Curr. Rheumatol. Rep. 2011 April; 13(2):123-131.
8. Finckh A., Gabay C. At the horizon of innovative therapy in rheumatology: new biologic agents. Curr. Opin. Rheumatol. 2008; 20: 269–75.
9. Moll M, Kuemmerle-Deschner JB Inflammasome and cytokine blocking strategies in autoinflammatory disorders. Clin. Immunol. 2013 147(3): 242-75.
10. LachmannH.J, Kone-PautI, Kuemmerle-Deschner J.B. etal. Use of Canakinumab in the Cryopyrin-Associated Periodic Syndrome. N. Engl. J. Med. 2009; 360(23): 2416-25.
11. Wulffraat NM, P Woo. Canakinumab in pediatric rheumatic diseases. Exp. Opin. Biol. Ther. 2013; 13(4):18-22.
12. Dinarello C.A. Bloking IL-1 in systemic inflammation. J. Exp. Med. 2005; 201(9):1355-9
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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