Is there any difference regarding atopy between children with familial Mediterranean fever and healthy controls?

INTRODUCTION: There are only a few studies regarding the prevalence of atopy in Familial Mediterranean fever (FMF) patients, and their results are conflicting. METHODS: In this study children with the diagnosis of FMF were evaluated for the presence of atopy by comparing with controls. One hundred and eighteen children diagnosed as FMF and 50 healthy age and sex matched controls were enrolled. They were evaluated for the presence of rhinitis, atopic dermatitis, urticaria and asthma. Laboratory assessment was done by measuring IgA, IgM, IgG, IgE levels, total eosinophil count and by performing skin prick test (SPT) panels for common allergens to children with FMF and healthy controls. RESULTS: One hundred and eighteen children (61girls and 57 boys) diagnosed as FMF with a median age of 120±47 months (range 36-204 months) were compared with 50 healthy controls (31 girls and 19 boys) having a median age of 126±37 (range 48-192 months). The mean percentage of total eosinophil count of patients was similar to that of the control group. The mean level of IgE was significantly higher in children with FMF than controls (136±268, 87±201, respectively; p values <0.05). The percentage of skin prick test positivity was similar for both patients and controls (13% and 8.2%, respectively; p>0.05). The prevalences of atopic dermatitis, allergic rhinitis, and asthma in the patient group were 5.08%, 28.8%, and 15.25%, respectively, while the control group had the prevalences of 0%, 36%, and 14% respectively. CONCLUSION: Children with FMF did not show an increase of atopic dermatitis, allergic rhinitis and asthma with respect to controls.

MEFV mutations in systemic onset juvenile idiopathic arthritis.

OBJECTIVES: Autoinflammatory diseases constitute a large spectrum of monogenic diseases like FMF or cryopyrin-associated periodic syndromes (CAPS) and complex genetic trait diseases such as systemic onset juvenile idiopathic arthritis (SoJIA). An increased rate of MEFV mutations has been shown among patients with PAN and HSP, in populations where FMF is frequent. The aim of the study is to search for MEFV mutations in our patients with SoJIA and see whether these mutations had an effect on disease course or complications. METHODS: Thirty-five children with the diagnosis of SoJIA were screened for 12 MEFV mutations. The control data were obtained from a previous study of our centre determining the carrier frequency in Turkish population. RESULTS: Two patients were homozygous and three patients were heterozygous for the M694V mutation. One patient was a compound heterozygote for the M680I/V726A mutations. Heterozygous V726A mutation was found in one patient. The overall mutation frequency of patients was 14.28%. This figure had been compared with the previously published rate of disease-causing mutations in this country, which is 5%. Disease-causing mutations were found to be significantly more frequent in the SoJIA patients than the population (P < 0.01). Among these, M694V was the leading mutation with a frequency of 10% in SoJIA. Six patients carrying MEFV mutations were among the most resistant cases requiring biological therapy. CONCLUSION: SoJIA patients had a significantly higher frequency of MEFV mutations but clinical studies with large number of patients are needed to confirm the association of MEFV mutations with SoJIA and its course.