Raynaud's phenomenon in pediatric age

Background

Raynaud's phenomenon (RP) is characterized by changes in the color of extremities: pallor, cyanosis and erythema. It´s prevalence in adults ranges between 3-20% but there are few studies in pediatric age. RP in boys or in children less than 12 years seems more associated to the secondary form.

Aim

Assessment of RP in children and adolescents referred to Centro Hospitalar Porto, in the last 11 years.

Methods

Retrospective analysis of clinical files of children and adolescents in which RP was the reason for consultation. Statistic analyses: SPSS version 19.

Results

Sixty six cases (71,2% female) met the inclusion criteria. Average age of RP onset was 12,5y with median duration of 3,6y. RP was associated to: erythema pernio (34,8%), inter-phalangeal joints pain (30.3%), hands edema (28,8%), acrocyanosis (18,2%), profuse sweating (12,1% ) and trophic changes of fingers pulp (10,6%.). Antinuclear antibodies (ANA) were titled in 64 patients (97%), being positive (>1/80) in ten (15.6%). Capillaroscopy was performed on 43 (65.2%): 32 (74,4%) minor changes (tortuosity and segmental enlargement), four (9,3%) significant changes (giant capillaries, hemorrhagic and avascular areas), seven (16,3%) normal.

Of 43 patients with complete study, five (11,6%) had secondary RP: two with juvenile systemic sclerosis, two in characterization and a probable case of mixed connective tissue disease.

Secondary RP occurred mostly in less that 12-years-old child. ANA ≥ 1/320 and significant changes on capillaroscopy had a significant statistic relationship with secondary RP.

Conclusion

RP can occur in children and, as in adults, in most cases is primary. ANA´s positivity and changes in capillary bed were predictors of secondary RP.