Granulomatosis with polyangiitis epidemiology and demographics: Difference between revisions

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{{Wegener's granulomatosis}}
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==Overview==
==Overview==
The prevalence of Granulomatosis with polyangiitis in Europe ranges from 23.7 to 160 per million person a year, with higher prevalence rates found in northern European countries such as Norway, southern Sweden, northern Germany and the United Kingdom. The [[incidence]] of the disease is 2.4 cases per million per year, with higher incidences found in Norway, Sweden, Germany, and the United Kingdom.<ref name="pmid24932888">{{cite journal| author=Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A et al.| title=Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. | journal=Semin Arthritis Rheum | year= 2014 | volume= 44 | issue= 2 | pages= 202-7 | pmid=24932888 | doi=10.1016/j.semarthrit.2014.05.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24932888  }} </ref>
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. The mean age of diagnosis of granulomatosis with polyangiitis is 58 years. The disease most commonly presents in patients that are either middle aged or elderly. The mean age of diagnosis in children is between the ages of 4 to 17. Males are more commonly affected with Granulomatosis with polyangiitis. The overall male to female ratio is approximately 1.2 to 1. In children females are more commonly seen with the disease. Granulomatosis with polyangiitis typically affects Caucasians.  


==Epidemiology==
==Epidemiology==
===Incidence===
* The incidence of Granulomatosis with polyangiitis in Europe ranges from 3 to 14.4 per million per year.<ref name="pmid24932888">{{cite journal| author=Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A et al.| title=Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. | journal=Semin Arthritis Rheum | year= 2014 | volume= 44 | issue= 2 | pages= 202-7 | pmid=24932888 | doi=10.1016/j.semarthrit.2014.05.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24932888  }} </ref>
* The incidence of Granulomatosis with polyangiitis in children ranges form 0.03 to 3.2 per 100,000 per year.<ref name="pmid19877069">{{cite journal| author=Cabral DA, Uribe AG, Benseler S, O'Neil KM, Hashkes PJ, Higgins G et al.| title=Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. | journal=Arthritis Rheum | year= 2009 | volume= 60 | issue= 11 | pages= 3413-24 | pmid=19877069 | doi=10.1002/art.24876 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19877069  }} </ref>


===Prevalence===
===Prevalence===
The prevalence of Granulomatosis with polyangiitis in Europe ranges from 23.7 to 160 per million persons.<ref name="pmid24932888">{{cite journal| author=Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A et al.| title=Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. | journal=Semin Arthritis Rheum | year= 2014 | volume= 44 | issue= 2 | pages= 202-7 | pmid=24932888 | doi=10.1016/j.semarthrit.2014.05.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24932888  }} </ref>
* The prevalence of Granulomatosis with polyangiitis in Europe ranges from 23.7 to 160 per million persons.<ref name="pmid24932888">{{cite journal| author=Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A et al.| title=Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. | journal=Semin Arthritis Rheum | year= 2014 | volume= 44 | issue= 2 | pages= 202-7 | pmid=24932888 | doi=10.1016/j.semarthrit.2014.05.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24932888  }} </ref>


===Incidence===
* The prevalence in the United States is 3 per 100,000 persons.<ref name="pmid8546743">{{cite journal| author=Cotch MF, Hoffman GS, Yerg DE, Kaufman GI, Targonski P, Kaslow RA| title=The epidemiology of Wegener's granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. | journal=Arthritis Rheum | year= 1996 | volume= 39 | issue= 1 | pages= 87-92 | pmid=8546743 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8546743  }}</ref><ref name="pmid25076302">{{cite journal| author=Muller K, Lin JH| title=Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings. | journal=Arch Pathol Lab Med | year= 2014 | volume= 138 | issue= 8 | pages= 1110-4 | pmid=25076302 | doi=10.5858/arpa.2013-0006-RS | pmc=4140401 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25076302  }}</ref>
The incidence of Granulomatosis with polyangiitis in Europe ranges from 3 to 14.4 per million per year.<ref name="pmid24932888">{{cite journal| author=Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A et al.| title=Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. | journal=Semin Arthritis Rheum | year= 2014 | volume= 44 | issue= 2 | pages= 202-7 | pmid=24932888 | doi=10.1016/j.semarthrit.2014.05.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24932888  }} </ref>
The incidence of Granulomatosis with polyangiitis in children is around .6 per million per year.<ref name="pmid23956873">{{cite journal| author=Tahghighi F, Moradinejad MH, Aghighi Y, Shiari R, Raeeskarami SR, Salehzadeh F et al.| title=Evaluation of 10-year experience of Wegener's granulomatosis in Iranian children. | journal=ISRN Rheumatol | year= 2013 | volume= 2013 | issue= | pages= 694928 | pmid=23956873 | doi=10.1155/2013/694928 | pmc=3728496 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23956873  }} </ref>


==Demographics==
==Demographics==
The age, sex, and race distribution of Granulomatosis with polyangiitis is dictated by the prevalence of the underlying cause within those demographic groups.
The rate distribution of Granulomatosis with polyangiitis varies upon age, sex, and race.


===Age===
===Age===
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.<ref name="pmid24932888">{{cite journal| author=Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A et al.| title=Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. | journal=Semin Arthritis Rheum | year= 2014 | volume= 44 | issue= 2 | pages= 202-7 | pmid=24932888 | doi=10.1016/j.semarthrit.2014.05.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24932888  }} </ref>
* Patients of all age groups may develop Granulomatosis with polyangiitis.
The disease most commonly presents in patients that are either middle aged or elderly.<ref name="pmid11083271">{{cite journal| author=Koldingsnes W, Nossent H| title=Epidemiology of Wegener's granulomatosis in northern Norway. | journal=Arthritis Rheum | year= 2000 | volume= 43 | issue= 11 | pages= 2481-7 | pmid=11083271 | doi=10.1002/1529-0131(200011)43:11<2481::AID-ANR15>3.0.CO;2-6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11083271  }} </ref>  
 
<ref name="pmid10693883">{{cite journal| author=Watts RA, Lane SE, Bentham G, Scott DG| title=Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. | journal=Arthritis Rheum | year= 2000 | volume= 43 | issue= 2 | pages= 414-9 | pmid=10693883 | doi=10.1002/1529-0131(200002)43:2<414::AID-ANR23>3.0.CO;2-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10693883  }} </ref> <ref name="pmid12794795">{{cite journal| author=Gonzalez-Gay MA, Garcia-Porrua C, Guerrero J, Rodriguez-Ledo P, Llorca J| title=The epidemiology of the primary systemic vasculitides in northwest Spain: implications of the Chapel Hill Consensus Conference definitions. | journal=Arthritis Rheum | year= 2003 | volume= 49 | issue= 3 | pages= 388-93 | pmid=12794795 | doi=10.1002/art.11115 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12794795  }} </ref>  
* The mean age of diagnosis of granulomatosis with polyangiitis is 58 years.
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.<ref name="pmid19877069">{{cite journal| author=Cabral DA, Uribe AG, Benseler S, O'Neil KM, Hashkes PJ, Higgins G et al.| title=Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. | journal=Arthritis Rheum | year= 2009 | volume= 60 | issue= 11 | pages= 3413-24 | pmid=19877069 | doi=10.1002/art.24876 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19877069  }} </ref>
* The incidence of the disease in female patients is 70 to 79 years of age and in male patients older than 80 years.<ref name="pmid24932888">{{cite journal| author=Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A et al.| title=Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. | journal=Semin Arthritis Rheum | year= 2014 | volume= 44 | issue= 2 | pages= 202-7 | pmid=24932888 | doi=10.1016/j.semarthrit.2014.05.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24932888  }} </ref>
* The disease most commonly presents in patients that are either middle aged or elderly.<ref name="pmid11083271">{{cite journal| author=Koldingsnes W, Nossent H| title=Epidemiology of Wegener's granulomatosis in northern Norway. | journal=Arthritis Rheum | year= 2000 | volume= 43 | issue= 11 | pages= 2481-7 | pmid=11083271 | doi=10.1002/1529-0131(200011)43:11<2481::AID-ANR15>3.0.CO;2-6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11083271  }} </ref><ref name="pmid10693883">{{cite journal| author=Watts RA, Lane SE, Bentham G, Scott DG| title=Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. | journal=Arthritis Rheum | year= 2000 | volume= 43 | issue= 2 | pages= 414-9 | pmid=10693883 | doi=10.1002/1529-0131(200002)43:2<414::AID-ANR23>3.0.CO;2-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10693883  }} </ref><ref name="pmid12794795">{{cite journal| author=Gonzalez-Gay MA, Garcia-Porrua C, Guerrero J, Rodriguez-Ledo P, Llorca J| title=The epidemiology of the primary systemic vasculitides in northwest Spain: implications of the Chapel Hill Consensus Conference definitions. | journal=Arthritis Rheum | year= 2003 | volume= 49 | issue= 3 | pages= 388-93 | pmid=12794795 | doi=10.1002/art.11115 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12794795  }} </ref>  
* 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.<ref name="pmid19877069">{{cite journal| author=Cabral DA, Uribe AG, Benseler S, O'Neil KM, Hashkes PJ, Higgins G et al.| title=Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. | journal=Arthritis Rheum | year= 2009 | volume= 60 | issue= 11 | pages= 3413-24 | pmid=19877069 | doi=10.1002/art.24876 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19877069  }} </ref>


It is rare in children, but has been seen in infants as young as 3 months old.
===Gender===
* Males are more commonly affected with Granulomatosis with polyangiitis.
* The overall male to female ratio is approximately 1.2 to 1.<ref name="pmid24932888">{{cite journal| author=Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A et al.| title=Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. | journal=Semin Arthritis Rheum | year= 2014 | volume= 44 | issue= 2 | pages= 202-7 | pmid=24932888 | doi=10.1016/j.semarthrit.2014.05.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24932888  }} </ref>
* In children females are more commonly seen with the disease.<ref name="pmid24891844">{{cite journal| author=Bohm M, Gonzalez Fernandez MI, Ozen S, Pistorio A, Dolezalova P, Brogan P et al.| title=Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis). | journal=Pediatr Rheumatol Online J | year= 2014 | volume= 12 | issue=  | pages= 18 | pmid=24891844 | doi=10.1186/1546-0096-12-18 | pmc=4041043 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24891844  }} </ref>


===Race===
===Race===
Granulomatosis with polyangiitis usually affects individuals of the Caucasian race. Other races are less likely to develop Granulomatosis with polyangiitis.
* Granulomatosis with polyangiitis typically affects Caucasians.  
<ref name="pmid24932888">{{cite journal| author=Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A et al.| title=Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. | journal=Semin Arthritis Rheum | year= 2014 | volume= 44 | issue= 2 | pages= 202-7 | pmid=24932888 | doi=10.1016/j.semarthrit.2014.05.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24932888  }} </ref>
* Other races can be affected, however the rate is lower than that of Caucasians.<ref name="pmid24932888">{{cite journal| author=Catanoso M, Macchioni P, Boiardi L, Manenti L, Tumiati B, Cavazza A et al.| title=Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study. | journal=Semin Arthritis Rheum | year= 2014 | volume= 44 | issue= 2 | pages= 202-7 | pmid=24932888 | doi=10.1016/j.semarthrit.2014.05.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24932888  }} </ref><ref name="pmid17553910">{{cite journal| author=Mohammad AJ, Jacobsson LT, Mahr AD, Sturfelt G, Segelmark M| title=Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden. | journal=Rheumatology (Oxford) | year= 2007 | volume= 46 | issue= 8 | pages= 1329-37 | pmid=17553910 | doi=10.1093/rheumatology/kem107 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17553910  }} </ref>
<ref name="pmid17553910">{{cite journal| author=Mohammad AJ, Jacobsson LT, Mahr AD, Sturfelt G, Segelmark M| title=Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden. | journal=Rheumatology (Oxford) | year= 2007 | volume= 46 | issue= 8 | pages= 1329-37 | pmid=17553910 | doi=10.1093/rheumatology/kem107 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17553910  }} </ref>


==References==
==References==

Latest revision as of 18:41, 27 March 2018

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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. The mean age of diagnosis of granulomatosis with polyangiitis is 58 years. The disease most commonly presents in patients that are either middle aged or elderly. The mean age of diagnosis in children is between the ages of 4 to 17. Males are more commonly affected with Granulomatosis with polyangiitis. The overall male to female ratio is approximately 1.2 to 1. In children females are more commonly seen with the disease. Granulomatosis with polyangiitis typically affects Caucasians.

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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