Scleroderma epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [2]

Overview

The majority of cases of scleroderma have been reported from the United States. The prevalence of scleroderma is approximately 24 cases per 100,000 individuals in the United States. Scleroderma commonly affects individuals between 20 to 50 years of age. Choctaw native Americans have a much higher prevalence of scleroderma than the general population. Females are more commonly affected than males. Familial clustering of scleroderma has been reported in United States and Australia.

Epidemiology and Demographics

Incidence

  • The incidence of scleroderma is approximately 0.23 to 2.28 cases per 100,000 individuals worldwide.[1]
  • The incidence of scleroderma is approximately 2 per 100,000 individuals in the United States.[2]

Prevalence

  • The prevalence of scleroderma is approximately 5 to 30 cases per 100,000 individuals worldwide.[1]
  • In 1990, the prevalence of scleroderma was estimated to be 27.6 cases per 100,000 individuals in the united states.[1]
  • The prevalence of scleroderma is approximately 24 cases per 100,000 individuals in the United States.[2]

Standardized mortality ratio

Age

  • Scleroderma commonly affects individuals between 20 to 50 years of age.[4]
  • Scleroderma may also occur in patients younger than 20 years or older than 50 years.

Race

  • Choctaw native Americans from southeastern Oklahoma were reported to have much higher prevalence of scleroderma than the general population.[5]
  • Scleroderma usually affects individuals of the African-American race more than the caucasian race.[6][7]

Gender

  • Females are more commonly affected by scleroderma than males. The female to male ratio is approximately ranging from 3:1 to 14:1.[7][8]

Region

  • Scleroderma cases are more frequently reported in the United States than in continental Europe.[2]
  • Familial clustering of scleroderma has been reported in United States and Australia.

References

  1. 1.0 1.1 1.2 Chifflot H, Fautrel B, Sordet C, Chatelus E, Sibilia J (February 2008). "Incidence and prevalence of systemic sclerosis: a systematic literature review". Semin. Arthritis Rheum. 37 (4): 223–35. doi:10.1016/j.semarthrit.2007.05.003. PMID 17692364.
  2. 2.0 2.1 2.2 Mayes MD (May 2003). "Scleroderma epidemiology". Rheum. Dis. Clin. North Am. 29 (2): 239–54. PMID 12841293.
  3. Ioannidis JP, Vlachoyiannopoulos PG, Haidich AB, Medsger TA, Lucas M, Michet CJ, Kuwana M, Yasuoka H, van den Hoogen F, Te Boome L, van Laar JM, Verbeet NL, Matucci-Cerinic M, Georgountzos A, Moutsopoulos HM (January 2005). "Mortality in systemic sclerosis: an international meta-analysis of individual patient data". Am. J. Med. 118 (1): 2–10. doi:10.1016/j.amjmed.2004.04.031. PMID 15639201.
  4. Alba MA, Velasco C, Simeón CP, Fonollosa V, Trapiella L, Egurbide MV, Sáez L, Castillo MJ, Callejas JL, Camps MT, Tolosa C, Ríos JJ, Freire M, Vargas JA, Espinosa G (March 2014). "Early- versus late-onset systemic sclerosis: differences in clinical presentation and outcome in 1037 patients". Medicine (Baltimore). 93 (2): 73–81. doi:10.1097/MD.0000000000000018. PMC 4616306. PMID 24646463.
  5. Arnett FC, Howard RF, Tan F, Moulds JM, Bias WB, Durban E, Cameron HD, Paxton G, Hodge TJ, Weathers PE, Reveille JD (August 1996). "Increased prevalence of systemic sclerosis in a Native American tribe in Oklahoma. Association with an Amerindian HLA haplotype". Arthritis Rheum. 39 (8): 1362–70. PMID 8702445.
  6. Reveille JD (April 2003). "Ethnicity and race and systemic sclerosis: how it affects susceptibility, severity, antibody genetics, and clinical manifestations". Curr Rheumatol Rep. 5 (2): 160–7. PMID 12628048.
  7. 7.0 7.1 Mayes MD, Lacey JV, Beebe-Dimmer J, Gillespie BW, Cooper B, Laing TJ, Schottenfeld D (August 2003). "Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population". Arthritis Rheum. 48 (8): 2246–55. doi:10.1002/art.11073. PMID 12905479.
  8. "Systemic scleroderma - Genetics Home Reference".

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