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Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.

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Cryopyrin-associated periodic syndrome epidemiology and demographics

Introduction

Overview

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

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

  • There is no racial predilection to cryopyrin-associated periodic syndrome.[4]

Gender

  • Cryopyrin-associated periodic syndrome affects men and women equally.[4]

Region

  • The majority of CAPS cases are reported in Europe.[3]
References

References

  1. ↑ 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.
  2. ↑ 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. ↑ 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. ↑ 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.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]