Rheumatic fever in children younger than 5 years: is the presentation different?

To review our experience with children who presented with rheumatic fever (RF) before 5 years of age and to compare their presentation with that of older children.

The cardiology database was reviewed to identify patients who were younger than 5 years and had a diagnosis RF using the Jones criteria from January 1985 through March 2000. Patient age, sex, date and age at presentation, and the major Jones criteria fulfilled were noted. When carditis was present, its severity was judged to be moderate to severe when there was radiographic cardiomegaly and/or clinical congestive heart failure. The clinical presentation of patients who presented in the first 5 years of life were compared with the presentation of those whose RF was diagnosed after 5 years of age. Clinical findings at follow-up evaluation and echocardiographic findings both at presentation and at follow-up were noted for the children who were younger than 5 years at presentation.

Of 541 cases of RF seen from January 1985 through March 20000, 27 (5%) were in children who were younger than 5 years (median: 4.0 years; range: 1.9-4.9 years). Major Jones criteria at presentation were arthritis in 17, carditis in 14, chorea in 3, and erythema marginatum in 3. The carditis was mild in 4 and moderate to severe in 10 patients. Compared with older children, younger children were more likely to present with moderate to severe carditis, arthritis without carditis or chorea, or the rash of erythema marginatum and were less likely to have chorea. The incidence of carditis was similar in the 2 groups as was the ratio of boys to girls. At follow-up (9.6 +/- 5.6 years), 69% of younger children who presented with carditis have clinical rheumatic heart disease. Subclinical, echocardiographically detected valvular abnormalities were detected both at presentation (33% of all children with RF before 5 years of age) and at follow-up (55% of those who initially had carditis).

Approximately 5% of children with RF were younger than 5 years at diagnosis. Compared with older patients, children who presented before 5 years of age were more likely to have moderate to severe carditis and to present with arthritis or the rash of erythema marginatum and were less likely to have chorea. Chronic rheumatic heart disease was common in young children who presented with carditis. Long-term follow-up is necessary to determine the outcome for young children with subclinical echocardiographic evidence of valvular disease.