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Systemic sclerosis in Iceland. A nationwide epidemiological study
A J Geirsson1, K Steinsson, S Guthmundsson
1Department of Internal Medicine, Landspitalinn, University Hospital, Reykjavík, Iceland.
Annals of the Rheumatic Diseases
|August 1, 1994
Summary
This study found a low incidence of systemic sclerosis (SS) in Iceland, with most patients exhibiting limited cutaneous involvement. Survival rates were high, and no HLA antigen association was identified.
Area of Science:
- Rheumatology
- Epidemiology
- Autoimmune Diseases
Background:
- Systemic sclerosis (SS) is a rare autoimmune disease characterized by fibrosis and vascular abnormalities.
- Understanding the epidemiology of SS is crucial for resource allocation and patient care.
Purpose of the Study:
- To determine the incidence, prevalence, and clinical characteristics of systemic sclerosis in Iceland.
- To provide updated epidemiological data on SS in a specific geographic region.
Main Methods:
- A retrospective study design was employed, including all patients diagnosed with SS in Iceland between 1975 and 1990.
- Data retrieval involved comprehensive searches of hospital and healthcare clinic registers, death records, and direct communication with physicians.
Main Results:
- The incidence of SS was found to be low, with rates of 0.7/100,000 for females and 0.05/100,000 for males.
- The age-standardized prevalence at the end of 1990 was 11.9/100,000 for females and 1.5/100,000 for males.
- A high proportion of patients (13 out of 18) had limited cutaneous systemic sclerosis, and five-year survival was 100%.
Conclusions:
- Iceland exhibits a low incidence of systemic sclerosis compared to previous surveys.
- The study highlights a significant proportion of patients with limited cutaneous involvement in the Icelandic SS population.
- Survival rates are favorable, with no identified HLA antigen associations.