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Systemic sclerosis in northwestern Spain: a 19-year epidemiologic study
Maria C Arias-Nuñez1, Javier Llorca, Tomas R Vazquez-Rodriguez
1From Divisions of Internal Medicine (MCA-N), Rheumatology (TRV-R, JAM-F, MAG-G), and Cardiology (CG-J), Hospital Xeral-Calde, Lugo; Division of Epidemiology and Computational Biology (JL, IG-A), School of Medicine, University of Cantabria, Santander; and Consejo Superior de Investigaciones Cientificas (JM), Granada, Spain.
Medicine
|September 17, 2008
Summary
This study on systemic sclerosis (SSc) in Spain found increasing incidence and prevalence rates over 19 years. Survival probability for SSc patients was significantly reduced, with cardiopulmonary issues being the primary cause of death.
Area of Science:
- Rheumatology and Epidemiology
- Systemic Sclerosis (SSc) disease patterns
Background:
- Systemic sclerosis (SSc) is a complex autoimmune disease with varying epidemiological characteristics globally.
- Understanding regional SSc incidence, prevalence, and clinical outcomes is crucial for public health planning and patient care.
Purpose of the Study:
- To determine the epidemiology of systemic sclerosis (SSc) in northwestern Spain.
- To assess SSc incidence, prevalence, clinical subtypes (diffuse SSc vs. limited SSc), and survival rates in the Lugo region.
- To establish a baseline for SSc in southern Europe and compare findings with other regions.
Main Methods:
- Retrospective cohort study of SSc patients diagnosed in Lugo, Spain, between 1988 and 2006.
- Utilized LeRoy and Medsger criteria and/or the 1980 American College of Rheumatology (ACR) preliminary classification criteria.
- Calculated age- and sex-adjusted incidence and prevalence rates; analyzed clinical characteristics and survival data.
Main Results:
- Overall age- and sex-adjusted incidence rate was 2.3 per 100,000 population (women: 3.5; men: 1.0).
- Incidence rates increased significantly over the study period, particularly in women and individuals aged 45+.
- Age-adjusted prevalence was 27.7 per 100,000 population; limited SSc (lSSc) was more common than diffuse SSc (dSSc).
Conclusions:
- The incidence and prevalence of SSc in northwestern Spain are comparable to those in Greece and parts of the US.
- A significant increase in SSc incidence was observed, especially in women, over the study duration.
- Patients with SSc face a significantly reduced survival probability, with cardiopulmonary complications being the leading cause of mortality.
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