Vitamin D linked to PFAPA syndrome

Objective: the etiology and pathogenesis of PFAPA is still unknown, the rapid response to corticosteroids treatment suggested an immune mechanism. The role of vitamin D in the development of immune mediated diseases is widely investigated. In this study we aimed to look for possible association between serum 25-hydroxy vitamin D levels and PFAPA syndrome. Method: Twenty-two patients diagnosed with PFAPA followed up at our private clinics between June 2010 and May 2012 were enrolled in the study in addition to 20 patients' age and gender-matched control group. All subjects had complete work-up medical examination and laboratory tests. Laboratory tests included complete blood count, C-reactive protein (CRP), throat culture, and vitamin D levels. Results: mean WBC and CRP values were significantly higher in the PFAPA group (WBC=13.9±2.3×10³ /mm² versus 5.8±3.4×10³ /mm² in the control group (P<0.05) and CRP 37.9±25.7mg/dl versus0.7±1.23mg/dl in the control group (P<0.05)). Vitamin D levels were significantly decreased in PFAPA group as compared with the control (12.27±11.02ng/ml in the PFAPA group versus 20.34±12.64ng/ml in the control group (P<0.05)). Multivariate analysis showed that CRP (odd ratio (OR)=3.0, 95% confidence interval (CI)=2.6-4.2, P=0.01 and serum vitamin D levels <30ng/ml OR=2.6, 95% CI=2.4-3.2, P=0.02 were associated with PFAPA. Conclusion: we found a significant correlation between PFAPA and vitamin D deficiency. Vitamin D deficiency can be significant risk factor for PFAPA occurrence.