Skip to main content
PMC home page
Annals of the Rheumatic Diseases logoLink to Annals of the Rheumatic Diseases
. 1994 Aug;53(8):502–505. doi: 10.1136/ard.53.8.502

Systemic sclerosis in Iceland. A nationwide epidemiological study.

A J Geirsson 1, K Steinsson 1, S Guthmundsson 1, V Sigurthsson 1
PMCID: PMC1005388  PMID: 7944633

Abstract

OBJECTIVES--To investigate the incidence, prevalence and clinical features of systemic sclerosis (SS) in Iceland. METHODS--All patients diagnosed with SS from 1975-90 were included. Retrieval for the study began in 1980 and was carried out by computerised search from registers of all hospitals and health care clinics and death registration files, and with personal communication with doctors in Iceland. RESULTS--Over a 16 year period from 1975-90, 15 new cases were found with an incidence of 0.7 and 0.05/100,000, for females and males at risk respectively, and 0.38 for both sexes. At the end of 1990 there were 18 patients alive with SS, 13 with limited and five with diffuse cutaneous involvement. The age standardised prevalence was 11.9 and 1.5/100,000 for females and males at risk respectively. The crude prevalence rate for both sexes was 7.1/100,000. There were five deaths, two patients died of SS related causes, one had SS renal disease. The relative risk of death was similar to that in the general population. The calculated five year survival rate was 100% and the 10 year survival rate 81%. No HLA antigen association was found. CONCLUSION--Compared with previous surveys this study shows a low incidence of systemic sclerosis and a high proportion of patients with limited cutaneous involvement.

Full text

PDF
502

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Asboe-Hansen G. Treatment of generalized scleroderma with inhibitors of connective tissue formation. Acta Derm Venereol. 1975;55(6):461–465. [PubMed] [Google Scholar]
  2. Asboe-Hansen G. Treatment of generalized scleroderma: updated results. Acta Derm Venereol. 1979;59(5):465–467. [PubMed] [Google Scholar]
  3. Barnett A. J. Scleroderma (progressive systemic sclerosis): progress and course based on a personal series of 118 cases. Med J Aust. 1978 Aug 12;2(4):129–134. doi: 10.5694/j.1326-5377.1978.tb131413.x. [DOI] [PubMed] [Google Scholar]
  4. Black C. M., Welsh K. I., Walker A. E., Bernstein R. M., Catoggio L. J., McGregor A. R., Jones J. K. Genetic susceptibility to scleroderma-like syndrome induced by vinyl chloride. Lancet. 1983 Jan 1;1(8314-5):53–55. doi: 10.1016/s0140-6736(83)91578-7. [DOI] [PubMed] [Google Scholar]
  5. COONS A. H. Fluorescent antibody methods. Gen Cytochem Methods. 1958;1:399–422. [PubMed] [Google Scholar]
  6. ERASMUS L. D. Scleroderma in goldminers on the Witwatersrand with particular reference to pulmonary manifestations. S Afr J Lab Clin Med. 1957 Sep;3(3):209–231. [PubMed] [Google Scholar]
  7. FARMER R. G., GIFFORD R. W., Jr, HINES E. A., Jr Prognostic significance of Raynaud's phenomenon and other clinical characteristics of systemic scleroderma. A study of 271 cases. Circulation. 1960 Jun;21:1088–1095. doi: 10.1161/01.cir.21.6.1088. [DOI] [PubMed] [Google Scholar]
  8. Gudmundsson S., Steinsson K. Systemic lupus erythematosus in Iceland 1975 through 1984. A nationwide epidemiological study in an unselected population. J Rheumatol. 1990 Sep;17(9):1162–1167. [PubMed] [Google Scholar]
  9. Hashemi S., Smith C. D., Izaguirre C. A. Anti-endothelial cell antibodies: detection and characterization using a cellular enzyme-linked immunosorbent assay. J Lab Clin Med. 1987 Apr;109(4):434–440. [PubMed] [Google Scholar]
  10. Maricq H. R., Weinrich M. C., Keil J. E., Smith E. A., Harper F. E., Nussbaum A. I., LeRoy E. C., McGregor A. R., Diat F., Rosal E. J. Prevalence of scleroderma spectrum disorders in the general population of South Carolina. Arthritis Rheum. 1989 Aug;32(8):998–1006. doi: 10.1002/anr.1780320809. [DOI] [PubMed] [Google Scholar]
  11. Medsger T. A., Jr, Masi A. T. Epidemiology of systemic sclerosis (scleroderma). Ann Intern Med. 1971 May;74(5):714–721. doi: 10.7326/0003-4819-74-5-714. [DOI] [PubMed] [Google Scholar]
  12. Medsger T. A., Jr, Masi A. T. Survival with scleroderma. II. A life-table analysis of clinical and demographic factors in 358 male U.S. veteran patients. J Chronic Dis. 1973 Oct;26(10):647–660. doi: 10.1016/0021-9681(73)90054-4. [DOI] [PubMed] [Google Scholar]
  13. Rocco V. K., Hurd E. R. Scleroderma and scleroderma-like disorders. Semin Arthritis Rheum. 1986 Aug;16(1):22–69. doi: 10.1016/0049-0172(86)90040-5. [DOI] [PubMed] [Google Scholar]
  14. Rowell N. R. The prognosis of systemic sclerosis. Br J Dermatol. 1976 Jul;95(1):57–60. doi: 10.1111/j.1365-2133.1976.tb15535.x. [DOI] [PubMed] [Google Scholar]
  15. Silman A. J. Epidemiology of scleroderma. Curr Opin Rheumatol. 1991 Dec;3(6):967–972. doi: 10.1097/00002281-199112000-00012. [DOI] [PubMed] [Google Scholar]
  16. Silman A. J. Epidemiology of scleroderma. Ann Rheum Dis. 1991 Nov;50 (Suppl 4):846–853. doi: 10.1136/ard.50.suppl_4.846. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. TUFFANELLI D. L., WINKELMANN R. K. Systemic scleroderma, A clinical study of 727 cases. Arch Dermatol. 1961 Sep;84:359–371. doi: 10.1001/archderm.1961.01580150005001. [DOI] [PubMed] [Google Scholar]
  18. Tan E. M., Rodnan G. P., Garcia I., Moroi Y., Fritzler M. J., Peebles C. Diversity of antinuclear antibodies in progressive systemic sclerosis. Anti-centromere antibody and its relationship to CREST syndrome. Arthritis Rheum. 1980 Jun;23(6):617–625. doi: 10.1002/art.1780230602. [DOI] [PubMed] [Google Scholar]
  19. Williams J. M., Downey C., Thonar E. J. Increase in levels of serum keratan sulfate following cartilage proteoglycan degradation in the rabbit knee joint. Arthritis Rheum. 1988 Apr;31(4):557–560. doi: 10.1002/art.1780310415. [DOI] [PubMed] [Google Scholar]
  20. Wynn J., Fineberg N., Matzer L., Cortada X., Armstrong W., Dillon J. C., Kinney E. L. Prediction of survival in progressive systemic sclerosis by multivariate analysis of clinical features. Am Heart J. 1985 Jul;110(1 Pt 1):123–127. doi: 10.1016/0002-8703(85)90525-3. [DOI] [PubMed] [Google Scholar]

Articles from Annals of the Rheumatic Diseases are provided here courtesy of BMJ Publishing Group

RESOURCES