Raynaud’s Phenomenon in the Pediatric Age

Raynaud’s phenomenon (RP) in children may, as in adults, be primary (idiopathic) or secondary to rheumatic diseases, drugs, or other conditions. This chapter aims to present a practical approach to clinical assessment and treatment of children with RP, with supporting evidence from studies specific to the pediatric age group. As with adults, RP can be the presenting feature of connective tissue disease (CTD). However, risk factors for progression from RP symptoms to a defined CTD phenotype are less well defined in children. The differentiation between primary and secondary forms of RP is important. Those with primary RP have a good outcome and can be managed with non-pharmacological approaches and, where necessary, vasodilatory drugs, with reassurance to the child and family that this condition is relatively benign. Identification of a child with secondary RP or at high risk to progression to secondary RP based on history, examination, and investigations is important to allow more careful monitoring and follow-up, with the aim to detect evolving CTD early.