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家族性地中海热患者亚临床炎症的评估:与突变类型和发作状态的关系:一项回顾性研究。

Evaluation of subclinical inflammation in familial Mediterranean fever patients: relations with mutation types and attack status: a retrospective study.

作者信息

Kelesoglu Fatih Mehmet, Aygun Erhan, Okumus Nazli Kubra, Ersoy Ayşenur, Karapınar Edanur, Saglam Nesibe, Aydın Nur Gokce, Senay Beyza Betul, Gonultas Sumeyye, Sarisik Elif, Can Melike Zeynep, Atay Sirin, Basbug Dilruba, Tiryaki Feyza Kubra, Ozer Sena, Durmus Rana Berru, Orem Fatih, Atay Tugrul, Acar Ahmet, Yilmaz Yasin, Kaya Seyma, Ciftkaya Aylin, Sarac Zeynep, Makar Cagri Can, Saracoglu Basak, Dogdu Gafur, Omeroglu Rukiye Eker

机构信息

Department of Pediatric Rheumatology, Istanbul Medical School, Istanbul University, Millet Caddesi, Fatih, Istanbul, 34093, Turkey.

Department of Pediatrics, Haseki Education and Training Hospital, Vatan Caddesi, Istanbul, 34093, Turkey.

出版信息

Clin Rheumatol. 2016 Nov;35(11):2757-2763. doi: 10.1007/s10067-016-3275-0. Epub 2016 Apr 23.

Abstract

Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disease of childhood and adulthood. Development of systemic amyloidosis and frequent attack influence quality of life and survival. There is sporadic evidence indicating subclinical inflammation in patients with FMF. We aimed to assess subclinical inflammation using neutrophil to lymphocyte ratio (NLR), platelet to lymphocyte ratio (PLR), and C-reactive protein (CRP) in pediatric patients with FMF in the attack-free period. In this retrospective study, we reviewed the files of all FMF patients in our pediatric rheumatology outpatient clinic in a tertiary center and enrolled those with sufficient clinical and laboratory data. We also enrolled 73 controls. We grouped the patients according to being in attack period or attack-free period. We compared CRP, NLR, PLR, and WBC (white blood cell) levels between different mutations and polymorphisms. We also compared patients in the attack period with those in attack-free period. We enrolled 61 patients in attack period, 509 patients in attack-free period, and 73 controls. There was no difference between patients with different mutations with respect to NLR or PLR levels in the attack-free period. However, CRP levels were higher in patients with homozygous exon 10 mutations, especially those with homozygous M694V mutations compared with other mutations. However, CRP levels were mostly normal in these patients. Our data are against the reported fact that patients with FMF have higher NLR or PLR levels in attack-free periods. However, CRP levels were higher in the presence of homozygous exon 10 mutations (in particular homozygous M694V mutations).

摘要

家族性地中海热(FMF)是一种发生于儿童期和成年期的常染色体隐性自身炎症性疾病。系统性淀粉样变性的发展和频繁发作会影响生活质量和生存期。有零星证据表明FMF患者存在亚临床炎症。我们旨在利用中性粒细胞与淋巴细胞比值(NLR)、血小板与淋巴细胞比值(PLR)以及C反应蛋白(CRP)评估处于发作间期的儿童FMF患者的亚临床炎症。在这项回顾性研究中,我们查阅了一家三级中心儿科风湿病门诊所有FMF患者的病历,并纳入了那些有足够临床和实验室数据的患者。我们还纳入了73名对照者。我们根据患者处于发作期或发作间期进行分组。我们比较了不同突变和多态性之间的CRP、NLR、PLR和白细胞(WBC)水平。我们还比较了发作期患者与发作间期患者。我们纳入了61名发作期患者、509名发作间期患者和73名对照者。在发作间期,不同突变的患者在NLR或PLR水平方面没有差异。然而,与其他突变相比,纯合子外显子10突变患者的CRP水平更高,尤其是那些具有纯合子M694V突变的患者。然而,这些患者的CRP水平大多正常。我们的数据与已报道的FMF患者在发作间期NLR或PLR水平较高这一事实相悖。然而,存在纯合子外显子10突变(特别是纯合子M694V突变)时CRP水平更高。

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