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

IF 3 3区医学 Q1 PEDIATRICS

European Journal of Pediatrics Pub Date : 2025-04-07 DOI:10.1007/s00431-025-06091-y

Mehveş Işıklar Ekici, Elif Çelikel, Zahide Ekici Tekin, Vildan Güngörer, Cüneyt Karagöl, Melike Mehveş Kaplan, Nimet Öner, Merve Cansu Polat, Didem Öztürk, Emine Özçelik, Yasemin Uğur Es, Sultan Nilay Yoğun, Banu Çelikel Acar

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引用次数: 0

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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来源期刊

European Journal of Pediatrics 医学-小儿科

CiteScore

5.90

自引率

2.80%

发文量

367

审稿时长

3-6 weeks

期刊介绍： The European Journal of Pediatrics (EJPE) is a leading peer-reviewed medical journal which covers the entire field of pediatrics. The editors encourage authors to submit original articles, reviews, short communications, and correspondence on all relevant themes and topics. EJPE is particularly committed to the publication of articles on important new clinical research that will have an immediate impact on clinical pediatric practice. The editorial office very much welcomes ideas for publications, whether individual articles or article series, that fit this goal and is always willing to address inquiries from authors regarding potential submissions. Invited review articles on clinical pediatrics that provide comprehensive coverage of a subject of importance are also regularly commissioned. The short publication time reflects both the commitment of the editors and publishers and their passion for new developments in the field of pediatrics. EJPE is active on social media (@EurJPediatrics) and we invite you to participate. EJPE is the official journal of the European Academy of Paediatrics (EAP) and publishes guidelines and statements in cooperation with the EAP.