Granulomatosis with polyangiitis epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]Amandeep Singh M.D.[4]

Overview

The incidence of Granulomatosis with polyangiitis in children ranges form 0.03 to 3.2 per 100,000 per year. The prevalence in the United States is 3 per 100,000 persons.

Epidemiology

Incidence

  • The incidence of Granulomatosis with polyangiitis in Europe ranges from 3 to 14.4 per million per year.[1]
  • The incidence of Granulomatosis with polyangiitis in children ranges form 0.03 to 3.2 per 100,000 per year.[2]

Prevalence

  • The prevalence of Granulomatosis with polyangiitis in Europe ranges from 23.7 to 160 per million persons.[1]
  • The prevalence in the United States is 3 per 100,000 persons.[3][4]

Demographics

The rate distribution of Granulomatosis with polyangiitis varies upon age, sex, and race.

Age

  • Patients of all age groups may develop Granulomatosis with polyangiitis.
  • The mean age of diagnosis of granulomatosis with polyangiitis is 58 years.
  • The incidence of the disease in female patients is 70 to 79 years of age and in male patients older than 80 years.[1]
  • The disease most commonly presents in patients that are either middle aged or elderly.[5][6][7]
  • The mean age of diagnosis of Granulomatosis with polyangiitis in children is between the ages of 4 to 17. However, the disease is rarely seen in children.[2]

Gender

  • Males are more commonly affected with Granulomatosis with polyangiitis.
  • The overall male to female ratio is approximately 1.2 to 1.[1]
  • In children females are more commonly seen with the disease.[8]

Race

  • Granulomatosis with polyangiitis typically affects Caucasians.
  • Other races can be affected, however the rate is lower than that of Caucasians.[1][9]

References

  1. 1.0 1.1 1.2 1.3 1.4 Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A; et al. (2014). "Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study". Semin Arthritis Rheum. 44 (2): 202–7. doi:10.1016/j.semarthrit.2014.05.005. PMID 24932888.
  2. 2.0 2.1 Cabral DA, Uribe AG, Benseler S, O'Neil KM, Hashkes PJ, Higgins G; et al. (2009). "Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood". Arthritis Rheum. 60 (11): 3413–24. doi:10.1002/art.24876. PMID 19877069.
  3. Cotch MF, Hoffman GS, Yerg DE, Kaufman GI, Targonski P, Kaslow RA (1996). "The epidemiology of Wegener's granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources". Arthritis Rheum. 39 (1): 87–92. PMID 8546743.
  4. Muller K, Lin JH (2014). "Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings". Arch Pathol Lab Med. 138 (8): 1110–4. doi:10.5858/arpa.2013-0006-RS. PMC 4140401. PMID 25076302.
  5. Koldingsnes W, Nossent H (2000). "Epidemiology of Wegener's granulomatosis in northern Norway". Arthritis Rheum. 43 (11): 2481–7. doi:10.1002/1529-0131(200011)43:11<2481::AID-ANR15>3.0.CO;2-6. PMID 11083271.
  6. Watts RA, Lane SE, Bentham G, Scott DG (2000). "Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom". Arthritis Rheum. 43 (2): 414–9. doi:10.1002/1529-0131(200002)43:2<414::AID-ANR23>3.0.CO;2-0. PMID 10693883.
  7. Gonzalez-Gay MA, Garcia-Porrua C, Guerrero J, Rodriguez-Ledo P, Llorca J (2003). "The epidemiology of the primary systemic vasculitides in northwest Spain: implications of the Chapel Hill Consensus Conference definitions". Arthritis Rheum. 49 (3): 388–93. doi:10.1002/art.11115. PMID 12794795.
  8. Bohm M, Gonzalez Fernandez MI, Ozen S, Pistorio A, Dolezalova P, Brogan P; et al. (2014). "Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)". Pediatr Rheumatol Online J. 12: 18. doi:10.1186/1546-0096-12-18. PMC 4041043. PMID 24891844.
  9. Mohammad AJ, Jacobsson LT, Mahr AD, Sturfelt G, Segelmark M (2007). "Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden". Rheumatology (Oxford). 46 (8): 1329–37. doi:10.1093/rheumatology/kem107. PMID 17553910.

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