Cryopyrin-associated periodic syndrome epidemiology and demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]
Overview
The incidence cryopyrin-associated periodic syndrome is approximately 0.0343 per 100,000 individuals aged 16 years old or younger worldwide. The prevalence of cryopyrin-associated periodic syndrome is approximately 0.1 per 100,000 individuals worldwide. CAPS is usually first presented in the infancy. There is no racial predilection to cryopyrin-associated periodic syndrome. Cryopyrin-associated periodic syndrome affects men and women equally. The majority of CAPS cases are reported in Europe.
Epidemiology and Demographics
Incidence
- The incidence cryopyrin-associated periodic syndrome is approximately 0.0343 per 100,000 individuals aged 16 years old or younger worldwide.[1]
Prevalence
- The prevalence of cryopyrin-associated periodic syndrome is approximately 0.1 per 100,000 individuals worldwide.[2]
Age
- CAPS is usually first presented in the infancy. However, due to the vague nature of the symptom, diagnosis may be delayed until childhood or longer.[3]
Race
Gender
- Cryopyrin-associated periodic syndrome affects men and women equally.[4]
Region
- The majority of CAPS cases are reported in Europe.[3]
References
- ↑ Lainka, E.; Neudorf, U.; Lohse, P.; Timmann, C.; Bielak, M.; Stojanov, S.; Huss, K.; Kries, R. von; Niehues, T. (2010). "Analysis of Cryopyrin-Associated Periodic Syndromes (CAPS) in German Children: Epidemiological, Clinical and Genetic Characteristics". Klinische Pädiatrie. 222 (06): 356–361. doi:10.1055/s-0030-1265181. ISSN 0300-8630.
- ↑ Mehr, Sam; Allen, Roger; Boros, Christina; Adib, Navid; Kakakios, Alyson; Turner, Paul J; Rogers, Maureen; Zurynski, Yvonne; Singh-Grewal, Davinder (2016). "Cryopyrin-associated periodic syndrome in Australian children and adults: Epidemiological, clinical and treatment characteristics". Journal of Paediatrics and Child Health. 52 (9): 889–895. doi:10.1111/jpc.13270. ISSN 1034-4810.
- ↑ 3.0 3.1 Kastner, D. L. (2005). "Hereditary Periodic Fever Syndromes". Hematology. 2005 (1): 74–81. doi:10.1182/asheducation-2005.1.74. ISSN 1520-4391.
- ↑ 4.0 4.1 Cuisset, L.; Jeru, I.; Dumont, B.; Fabre, A.; Cochet, E.; Le Bozec, J.; Delpech, M.; Amselem, S.; Touitou, I. (2010). "Mutations in the autoinflammatory cryopyrin-associated periodic syndrome gene: epidemiological study and lessons from eight years of genetic analysis in France". Annals of the Rheumatic Diseases. 70 (3): 495–499. doi:10.1136/ard.2010.138420. ISSN 0003-4967.