Background/Purpose: Raynaud’s phenomenon (RP) is an episodic vasospasm of the peripheral arteries, causing pallor followed by cyanosis and redness with pain and sometimes paraesthesia or rarely ulceration of the fingers and toes. Primary RP (pRP) occurs without an underlying disease, while secondary RP occurs in association with an underlying connective tissue disease (CTD), mostly systemic lupus erythematous, juvenile dermatomyositis and systemic scleroderma. Predictors of a favorable outcome are still unraveled in pRP: the causative role of various autoantibodies remains to be elucidated mostly for pRP starting in childhood or adolescence. Our objective is to identify the potential predictors of outcome in a multicenter cohort of children and adolescents with pRP.

Methods: We performed a prospective data collection of demographic, clinical, laboratory and treatment features of 82 Italian children/adolescents with pRP (58 females, 24 males, median age at disease onset: 13.5 years, median age at diagnosis: 14.8 years), managed in 4 pediatric rheumatologic Units and 1 transition clinic during the last three years. Demographic characteristics included sex, age and ethnicity. The evaluation included clinical pictures, eventual disease associations, pubertal status, laboratory data and nailfold videocapillaroscopy (NVC) at baseline and at regular 6-month-follow-up. Laboratory examinations included erythrosedimentation rate, C-reactive protein, transaminases, serum creatinine, hemoglobin, complement fractions C4 and C3, renal and thyroid function and specific serum autoantibodies (anti-nuclear antibodies [ANA], anti-DNAds, anti-ENA, anti-cardiolipin, anti-Scl-70 and anti-centromere antibodies). Screening for coeliac disease was performed at the first evaluation. Treatment details included the eventual specific drug used, its dosage and overall treatment duration. Out of 82, 20 patients were treated with hydroxicloroquine, 10 with calcium blockers, 1 with low-dose aspirin, 3 with iloprost, while the remaining 48 did not receive any drug. A forward stepwise multiple logistic regression analysis was used to find any association among sex, pubertal status, inflammatory parameters, NVC abnormalities, all serum autoantibodies and the risk of developing a CTD at baseline and at 36-month-follow-up. The software used was STATA 10. A p-value <0.05 was considered significant.

Results: ANA positivity at baseline was significantly associated with the risk of developing a CTD (p <0.05). No NVC abnormalities was related to specific patients' outcome. No patient was positive at the screening for coeliac disease.

Conclusion: Our data show that ANA positivity appears as the only potential predictor of poor outcome and even progression to CTD in children and adolescents with pRP.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/primary-raynauds-phenomenon-in-a-multicenter-cohort-of-italian-children-and-adolescents-which-prognostic-relevance-for-serological-tests/