Background The pathogenesis of PFAPA syndrome is unknown. According to Stojanov et al. cytokine profile in PFAPA syndrome suggests Th1 mediated inflammatory process resulting in continuous inflammation and reduced Th2 anti-inflammatory response. Interferon γ might be responsible for suppressing the production of IL-4 and IL10. Hung et al. showed suppressive effects of ketotifen on the expression of Th1 and Th2 related chemokines of human monocytes.

The aim of this study was to assess autoimmune response following annual influenza vaccination in apparently healthy adults, staff at a children's hospital. 92 healthy adult subjects were tested for autoantibodies including antinuclear antibodies (ANA), anti-extractable nuclear antigen antibodies (anti-ENA), antiphospholipid antibodies (aPL), namely anticardiolipin antibodies (aCL), anti-beta2-glycoprotein I antibodies (aβ2-GPI) and lupus anticoagulant (LA). Blood samples were taken from each participant before annual influenza vaccination, one month and six months after vaccination. Before influenza vaccination 26% of participants were positive for ANA, 1% for anti-ENA, 16% for aCL, 7% for aβ2-GPI and 2% for LA. One month after influenza vaccination 76% of participants showed no change in autoantibodies titres. Six months after influenza vaccination 74% of participants showed no change in autoantibodies titres. Overall, there was no statistically significant difference in the percentage of positive ANA, aCL, aβ2-GPI and LA before and 6 months after the vaccination. Five participants developed autoantibodies 6 months after the vaccination and one who was initially low positive for ANA became highly positive (1:320). Eleven participants had only transiently increased autoantibodies. Persistently positive or progressively increased levels of autoantibodies during 6 months' follow up were observed in 6 persons (7%). Our study showed a high percentage of positive autoantibody testing among healthy adult staff at a children's hospital. There was no statistically significant difference in the percentage of positive autoantibodies before and after influenza vaccination. However, our study clearly demonstrated induction of autoantibodies production in selected subjects.

OBJECTIVE To determine the prevalence of anticardiolipin antibodies (aCL), anti-beta(2)-glycoprotein I (anti-beta(2)GPI) antibodies, and lupus anticoagulant (LAC) in a large cohort of children with systemic lupus erythematosus (SLE), and to evaluate the associations with neuropsychiatric manifestations. METHODS A single-center retrospective cohort study with longitudinal followup of antiphospholipid antibodies (aPL) in 137 children with SLE (25 boys and 112 girls, mean age at diagnosis 13.0 years) was performed. Patients were followed up for a mean of 31 months. RESULTS At the time of diagnosis, 65% of the children were aCL positive, 41% had anti-beta(2)GPI antibodies, and 26% were LAC positive. Analysis of the association between presence of aPL and individual neuropsychiatric manifestations at diagnosis showed a statistically significant association of positive LAC with cerebrovascular disease (5 patients; P = 0.015). A persistently positive aCL was observed in 50%, anti-beta(2)GPI antibodies in 29%, and LAC in 16% of children over time. The prevalence of anti-beta(2)GPI antibodies, but not aCL and LAC, was found to be statistically significantly higher in children with neuropsychiatric disease compared with those without (P = 0.02). Comparison for specific neuropsychiatric manifestations showed a statistically significant association between a persistently positive LAC and chorea (2 patients; P = 0.02). CONCLUSION The prevalence of anti-beta(2)GPI antibodies was found to be higher in the group of SLE patients with neuropsychiatric disease compared with those without. Our data suggest an association between LAC and cerebrovascular disease at the time of SLE diagnosis and chorea over the disease course, but not between aPL and other neuropsychiatric manifestations.

OBJECTIVE To use the Pediatric Rheumatology International Trials Organization (PRINTO) core set of outcome measures to develop a validated definition of improvement for the evaluation of response to therapy in juvenile systemic lupus erythematosus (SLE). METHODS Thirty-seven experienced pediatric rheumatologists from 27 countries, each of whom had specific experience in the assessment of juvenile SLE patients, achieved consensus on 128 patient profiles as being clinically improved or not improved. Using the physicians' consensus ratings as the gold standard measure, the chi-square, sensitivity, specificity, false-positive and false-negative rates, area under the receiver operating characteristic curve, and kappa level of agreement for 597 candidate definitions of improvement were calculated. Only definitions with a kappa value greater than 0.7 were retained. The top definitions were selected based on the product of the content validity score multiplied by its kappa statistic. RESULTS The definition of improvement with the highest final score was at least 50% improvement from baseline in any 2 of the 5 core set measures, with no more than 1 of the remaining worsening by more than 30%. CONCLUSION PRINTO proposes a valid and reproducible definition of improvement that reflects well the consensus rating of experienced clinicians and that incorporates clinically meaningful change in core set measures in a composite end point for the evaluation of global response to therapy in patients with juvenile SLE. The definition is now proposed for use in juvenile SLE clinical trials and may help physicians to decide whether a child with SLE responded adequately to therapy.