Incidence of pediatric rheumatic diseases in a regional population in Austria

To establish a population based disease registry for pediatric rheumatology in a defined population of Austria; to describe the demographic and diagnostic classification of children referred to pediatric rheumatology clinics; and to estimate the incidence of pediatric rheumatic diseases in Eastern Austria.

For 2 years (1997-98) all pediatric rheumatology centers in the area contributed data on all new cases to a prospective multicenter patient registry. Diagnostic criteria defined the rheumatic disease cases, determined by a pediatric rheumatologist, and record linkage was carried out to avoid duplication of subjects.

Rheumatic conditions were diagnosed in 107 subjects. Juvenile rheumatoid arthritis (JRA) was the most frequently encountered rheumatic condition (49.5%), followed by spondyloarthropathy (SpA, 33.6%) and systemic lupus erythematosus (SLE, 5.6%). The mean annual incidence of JRA, SpA, and SLE among children referred to pediatric rheumatology centers was 4.28, 2.9, and 0.48 per 100,000 children at risk, respectively.

Establishment of a population based disease registry led to collection of descriptive epidemiologic data on a defined regional cohort of children with rare disorders. Our registry will provide data on pediatric rheumatic diseases in a European population and will allow more accurate comparisons between populations for future research. Our data also indicate that more resources should be designated for the care of pediatric rheumatic diseases in view of the relatively high incidences of these diseases.