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Incidence of systemic connective tissue diseases in children: a nationwide prospective study in Finland
P M Pelkonen1, H J Jalanko, R K Lantto
1Children's Hospital, University of Helsinki, Finland.
Insights
Systemic connective tissue diseases (CTD) and systemic onset juvenile arthritis (S-JA) are rare in children under 16. This study quantifies their low incidence in Finland, providing crucial epidemiological data.
Area of Science:
- Pediatric Rheumatology
- Epidemiology
- Immunology
Background:
- Systemic connective tissue diseases (CTD) and systemic onset juvenile arthritis (S-JA) are significant pediatric conditions.
- Understanding their incidence is crucial for public health and resource allocation.
Purpose of the Study:
- To determine the incidence of various systemic CTDs and S-JA in children aged 0-15 years.
- To provide updated epidemiological data on these rare pediatric rheumatic diseases.
Main Methods:
- A nationwide, prospective, hospital-based study in Finland over 4 years.
- Inclusion of data from the National Hospital Discharge Register for a population of 1.02 million.
- Application of established classification criteria for adult CTD and juvenile arthritis (JA).
Main Results:
- Annual incidence rates per 100,000: S-JA (0.47), systemic lupus erythematosus (0.37), polymyositis/dermatomyositis (PM/DM) (0.30), mixed connective tissue disease (0.10), scleroderma (0.05).
- Girls were more frequently affected in CTD and S-JA, while PM/DM showed an even sex distribution.
- CTD and S-JA are confirmed as rare in the pediatric population.
Conclusions:
- Systemic CTD and S-JA are infrequent in individuals under 16 years of age.
- CTD incidence is estimated at 4-7% of JA incidence.
- S-JA constitutes only 2-4% of all JA cases.
Objective:
To study the incidence of systemic connective tissue diseases (CTD) and of systemic onset juvenile arthritis (S-JA) in children aged 0 to 15 years.
Methods:
A nationwide, prospective, hospital based series of new patients from Finland collected during a 4-year period and supplemented with data from the National Hospital Discharge Register. The population at risk was 1.02 million. Classification criteria developed for adult CTD and the American Rheumatism Association criteria for JA were used.
Results:
The annual incidence rates found were as follows: systemic lupus erythematosus 0.37, polymyositis/dermatomyositis (PM/DM) 0.30, mixed connective tissue disease 0.10, scleroderma 0.05, and S-JA 0.47/100,000. Girls outnumbered boys in the whole series of CTD (24:9) and also in S-JA (11:8), but in PM/DM the sex distribution was even.
Conclusion:
CTD and S-JA are rare before the age of 16 years. From the present incidence figures and those recently published for JA the incidence of CTD is estimated to be between 4 and 7% of that of JA, and S-JA accounts for only 2 to 4% of all JA.
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