How familial Mediterranean fever affects the characteristics of immunoglobulin a vasculitis in pediatric patients at the time of diagnosis?

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Abstract
Familial Mediterranean fever (FMF) is the most common autoinflammatory disease of childhood and is frequently associated with systemic inflammatory diseases such as vasculitis. The aim of this study is to evaluate the impact of FMF on clinical and laboratory findings and disease activity of immunoglobulin A vasculitis (IgAV) at presentation. Patients with IgAV who were followed up for more than 3 months were included in the study. Patients were divided into two subgroups regarding presence of FMF. Demographic characteristics, clinical findings in the first 3 months, laboratory findings at the time of diagnosis, vasculitis activity scores (PVAS), and treatments administered were recorded. A total of 662 patients with IgAV were included in the study. FMF was diagnosed in 49 (7.4%) patients with IgAV. Patients with FMF had more gastrointestinal tract and renal involvement, higher PVAS score, higher C-reactive protein levels, and higher need for steroids, cyclophosphamide, and intravenous immunoglobulin (p = 0.01, p = 0.03, p < 0.001, p < 0.001, p = 0.04, p < 0.001, p = 0.01, respectively). When patients were analyzed according to MEFV mutations, the effect of FMF on vasculitis was more prominent especially in patients carrying homozygous and compound heterozygous mutations in exon 10. Conclusion: FMF may lead to more severe clinical and laboratory findings and disease activity at the time of IgAV diagnosis. The clinician should be aware that the course of IgAV may be affected by the presence of FMF. Trial registration: E2-24–6125, 21.01.2024, retrospectively registered. What is Known:• In patients with familial Mediterranean fever (FMF), some vasculitides are more common and may affect the course of vasculitis. What is New:• At the time of immunoglobulin A vasculitis diagnosis, patients with FMF may have more system involvement, higher vasculitis activation scores, and acute phase reactants and require more intensive treatment than those without FMF.
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  • University of Health Sciences

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