Abstract

Familial Mediterranean fever (FMF) is A common periodic fever syndrome. The causative gene of the FMF is named Mediterranean Fever gene (MEFV). Increased inflammation in FMF may play a role as a trigger for the development of some diseases. The objective of the study is to evaluate the frequency of comorbid disorders in children followed up with diagnosis of FMF. Additionally, we aimed to assess the association between FMF and other inflammatory conditions in a large pediatric FMF cohort. A total of 686 FMF patients were included in the cross-sectional study. A questionnaire including questions about characteristics of fever episodes, presence of arthralgia, arthritis, abdominal pain, chest pain during and co-existence of any other disease diagnosed by a physician was filled out by face-to-face interviews with patients or their parents. Female–male ratio was 0.85. Median age at the time of study, age at disease onset and at the time of diagnosis were 12.9 (1.7–22.3), 3 (0.08–17), and 6 (0.75–17) years, respectively. In 130 (18.9%) FMF patients we detected co-existing inflammatory condition. The most common co-existing diseases were: juvenile idiopathic arthritis 42 (6.1%), asthma/reactive airway disease 29 (4.2%), Henoch–Schönlein purpura 20 (2.9%), uveitis 12 (1.7%) and inflammatory bowel disease 10 (1.4%). Except for asthma/reactive airway disease and inflammatory bowel disease, there was no significant difference regarding the type of MEFV gene mutation. We have reported increased frequencies of various inflammatory conditions and decreased frequency of asthma in patients with FMF.

中文翻译：

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家族性地中海热儿童并存疾病的评估

摘要

家族性地中海热（FMF）是一种常见的周期性发热综合征。FMF的致病基因被称为地中海热病基因（MEFV）。FMF中炎症的增加可能是某些疾病发展的诱因。该研究的目的是评估伴有FMF诊断的儿童合并症的发生频率。此外，我们旨在评估小儿FMF队列中FMF与其他炎症状况之间的关联。横断面研究共纳入686名FMF患者。通过与患者或其父母进行面对面访谈，填写了一份有关发烧发作，关节痛，关节炎，腹痛，胸痛以及医生诊断出的其他疾病并存的问题的问卷。男女比例为0.85。研究时，疾病发作时和诊断时的中位年龄分别为12.9（1.7–22.3），3（0.08–17），和6（0.75-17）年。在130名（18.9％）FMF患者中，我们检测到并存的炎症状态。最常见的并存疾病是：青少年特发性关节炎42（6.1％），哮喘/反应性气道疾病29（4.2％），过敏性紫癜20（2.9％），葡萄膜炎12（1.7％）和炎性肠病10 （1.4％）。除哮喘/反应性气道疾病和炎性肠病外，MEFV基因突变的类型没有显着差异。我们已经报道了FMF患者各种炎性疾病的发生频率增加，哮喘的发生频率降低。过敏性紫癜20（2.9％），葡萄膜炎12（1.7％）和炎性肠病10（1.4％）。除哮喘/反应性气道疾病和炎性肠病外，MEFV基因突变的类型没有显着差异。我们已经报道了FMF患者各种炎性疾病的发生频率增加，哮喘的发生频率降低。过敏性紫癜20（2.9％），葡萄膜炎12（1.7％）和炎性肠病10（1.4％）。除哮喘/反应性气道疾病和炎性肠病外，MEFV基因突变的类型没有显着差异。我们已经报道了FMF患者各种炎性疾病的发生频率增加，哮喘的发生频率降低。