Background: The periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, is an autoinflammatory disease characterised by regularly recurrent fever episodes, due to seemingly unprovoked inflammation.
Objective and hypotheses: The aim of the study was to assess serum 25-hydroxy cholecalciferol (25(OH)D) concentrations in children with PFAPA and to evaluate longitudinally the effect of wintertime supplementation on 25(OH)D status and the immune response in these children.
Method: We have evaluated 25 Italian patients (19 males, six females, aged 2.45.3 years) who fulfilled the Euro-Fever PFAPA criteria. For each patient, we recorded demographic and anthropometric data, clinical manifestations, serum calcium, phosphate, and 25(OH)D. After 400 UI 25(OH)D supplementation, clinical, and auxological characteristics, calcium and phosphate, and 25(OH)D concentrations were re-evaluated. Data were compared with a sex- and age-matched control group (74 males, 39 females, mean age 4.9±1.3 years, range 2.18.3 years).
Results: PFAPA patients showed very reduced 25(OH)D levels than controls (P<0.0001). Regarding the effect of the different seasons on 25(OH)D status, 25(OH)D levels in winter were significantly reduced in respect to those of the summer (P<0.005), these values were significantly lesser than controls (P<0.005). However, 25(OH)D levels correlated with episodes of fever (P<0.005) and CRP (P<0.005). After vitamin D3 supplementation, PFAPA patients displayed significantly increased 25(OH)D levels, showing a significative reduction of febrile episodes and its characteristics (mean duration of fever episodes, P<0.05 and number of febrile episodes for year, P<0.005).
Conclusion: Deficient and insufficient vitamin D serum levels were found in most children with PFAPA. Hypovitaminosis D can be significant risk factor for PFAPA recurrence. In fact, 25(OH)D supplementation seems significantly to reduce PFAPA episodes and fever duration, disclosing the importance of vitamin D as immunoregulatory factor in this syndrome.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology