OBJECTIVE:

To examine the clinical presentation and disease associations of Raynaud's phenomenon (RP) in children and adolescents.

METHODS:

A systematic retrospective chart review was conducted of 123 cases drawn from 2 computerized databases at the Children's Hospital of Boston. Participants aged <19 years with episodic reversible color changes in the extremities were examined. Case records were analyzed for clinical presentation, disease associations, and physical examination and laboratory findings.

RESULTS:

In contrast to the findings of smaller pediatric series reported to date, the large majority of our patients-approximately 70%-did not have a recognized underlying connective tissue disease. For both primary and secondary RP, approximately 80% of patients were female, and mean age of onset was similar in the 2 groups. Biphasic or triphasic color changes were less common than monophasic changes and were no more common in secondary than primary RP. Findings predictive of secondary RP were limited to the presence of antinuclear antibodies and abnormal nailfold capillaries. Antiphospholipid antibodies were found at some time in at least 21% of patients with both primary and secondary RP.

CONCLUSIONS:

RP in children, as in adults, principally affects girls and is frequently free of association with connective tissue disease. Antinuclear antibody positivity and abnormal nailfold capillaries correlate with secondary disease. Antiphospholipid antibodies are surprisingly common, a new finding of uncertain implications.