Pm. Pelkonen et al., INCIDENCE OF SYSTEMIC CONNECTIVE-TISSUE DISEASES IN CHILDREN - A NATIONWIDE PROSPECTIVE-STUDY IN FINLAND, Journal of rheumatology, 21(11), 1994, pp. 2143-2146
Objective. To study the incidence of systemic connective tissue diseas
es (CTD) and of systemic onset juvenile arthritis (S-JA) in children a
ged 0 to 15 years. Methods. A nationwide, prospective, hospital based
series of new patients from Finland collected during a 4-year period a
nd supplemented with data from the National Hospital Discharge Registe
r. The population at risk was 1.02 million. Classification criteria de
veloped for adult CTD and the American Rheumatism Association criteria
for JA were used. Results. The annual incidence rates found were as f
ollows: systemic lupus erythematosus 0.37, poly myositis/dermatomyosit
is (PM/DM) 0.30, mixed connective tissue disease 0.10, scleroderma 0.0
5, and S-JA 0.47/100,000. Girls outnumbered boys in the whole series o
f CTD (24:9) and also in S-JA (11:8), but in PM/DM the sex distributio
n was even. Conclusion. CTD and S-JA are rare before the age of 16 yea
rs. From the present incidence figures and those recently published fo
r 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.