Systemic sclerosis in Iceland. A nationwide epidemiological study.

A J Geirsson, K Steinsson, S Guthmundsson, V Sigurthsson

Department of Internal Medicine, Landspitalinn, University Hospital, Reykjavík, Iceland.

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.

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

• Hunzelmann, N., Genth, E., Krieg, T., Lehmacher, W., Melchers, I., Meurer, M., Moinzadeh, P., Muller-Ladner, U., Pfeiffer, C., Riemekasten, G., Schulze-Lohoff, E., Sunderkoetter, C., Weber, M., Worm, M., Klaus, P., Rubbert, A., Steinbrink, K., Grundt, B., Hein, R., Scharffetter-Kochanek, K., Hinrichs, R., Walker, K., Szeimies, R.-M., Karrer, S., Muller, A., Seitz, C., Schmidt, E., Lehmann, P., Foeldvari, I., Reichenberger, F., Gross, W.L., Kuhn, A., Haust, M., Reich, K., Bohm, M., Saar, P., Fierlbeck, G., Kotter, I., Lorenz, H.-M., Blank, N., Grafenstein, K., Juche, A., Aberer, E., Bali, G., Fiehn, C., Stadler, R., Bartels, V. (2008). The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology (Oxford) 47: 1185-1192 [Abstract] [Full Text]  
• TAGIL, M., DIETERICH, J., KOPYLOV, P. (2007). WOUND HEALING AFTER HAND SURGERY IN PATIENTS WITH SYSTEMIC SCLEROSIS--A RETROSPECTIVE ANALYSIS OF 41 OPERATIONS IN 19 PATIENTS. J Hand Surg Eur Vol 32: 316-319 [Abstract] [Full Text]  
• Walker, U A, Tyndall, A, Czirjak, L, Denton, C, Farge-Bancel, D, Kowal-Bielecka, O, Muller-Ladner, U, Bocelli-Tyndall, C, Matucci-Cerinic, M, EUSTAR Co-authors, (2007). Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database. Ann Rheum Dis 66: 754-763 [Abstract] [Full Text]  
• Le Guern, V., Mahr, A., Mouthon, L., Jeanneret, D., Carzon, M., Guillevin, L. (2004). Prevalence of systemic sclerosis in a French multi-ethnic county. Rheumatology (Oxford) 43: 1129-1137 [Abstract] [Full Text]  
• Lambe, M., Bjornadal, L., Neregard, P., Nyren, O., Cooper, G. S. (2004). Childbearing and the Risk of Scleroderma: A Population-based Study in Sweden. Am J Epidemiol 159: 162-166 [Abstract] [Full Text]  
• Geirsson, A J, Wollheim, F A, Akesson, A (2001). Disease severity of 100 patients with systemic sclerosis over a period of 14 years: using a modified Medsger scale. Ann Rheum Dis 60: 1117-1122 [Abstract] [Full Text]  
• Hesselstrand, R., Scheja, A., Åkesson, A. (1998). Mortality and causes of death in a Swedish series of systemic sclerosis patients. Ann Rheum Dis 57: 682-686 [Abstract] [Full Text]