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家族性地中海热发作期间患者会怎么做?他们的应对策略及相关因素。

What do patients do during a familial Mediterranean fever attack? Their strategies and associated factors.

作者信息

Kılıç Özlem, Çolak Seda, Tekgöz Emre, Kaya Mehmet Nur, Çınar Muhammet, Yılmaz Sedat

机构信息

Department of Rheumatology, Ankara Training and Research HospitalAnkara Training and Research Hospital, University of Health Sciences, Hacettepe, Ulucanlar Street, No:89, 06230, Altındağ/Ankara, Türkiye.

Department of Internal Medicine, Division of Rheumatology, Gülhane Training and Research Hospital, University of Health Sciences , Ankara, Türkiye.

出版信息

Intern Emerg Med. 2025 Jul 1. doi: 10.1007/s11739-025-04039-6.

Abstract

Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disease characterised by recurrent fever and serositis. Despite colchicine's proven efficacy, attacks may persist, necessitating the implementation of various mitigation strategies by patients.  To identify attack mitigation strategies and associated factors in FMF patients. A cross-sectional study at a tertiary rheumatology clinic included adults (≥ 18 years) meeting Tel-Hashomer criteria, on colchicine for ≥ 6 months, with ≥ 1 attack in the past six months. Patients were asked about their attack mitigation strategies, including colchicine dose increase, emergency department (ED) admission, resting, abdominal hot pack, dietary changes, on-demand anakinra, herbs, paracetamol, non-steroidal anti-inflammatory drugs (NSAIDs), and corticosteroid use. Among 258 patients (98 men, 160 women; median age 35 years, disease duration 18 years), 93% used mitigation strategies. The most common first choice was ED admission (19.8%), followed by colchicine dose increase (18.2%), resting (14.3%), dietary changes (4.3%), NSAIDs (7%), paracetamol (6.6%), on-demand anakinra (6.2%), herbs (6.2%), and corticosteroids (5%). Normal C-reactive protein (CRP) levels, shorter last attack duration, lower international Severity Score for FMF (ISSF), non-working and non-M694V mutations were linked with colchicine dose increase (p < 0.05). M694V positivity, working, persistent CRP elevation, dominant serositis and musculoskeletal attacks, education level ≤ 8 years, longer last attack duration, higher ISSF, current colchicine resistance and non-adherence were linked with ED admission (p < 0.05). Most FMF patients used strategies to mitigate attacks, mainly ED admission and colchicine dose increase. These were influenced by genetic mutations, attack type, working status, education, and CRP levels.

摘要

家族性地中海热(FMF)是一种常染色体隐性自身炎症性疾病,其特征为反复发热和浆膜炎。尽管秋水仙碱已被证实有效,但发作可能持续存在,这就需要患者采取各种缓解策略。为了确定FMF患者的发作缓解策略及相关因素。在一家三级风湿病诊所进行的一项横断面研究纳入了符合泰尔-哈绍默标准的成年人(≥18岁),服用秋水仙碱≥6个月,且在过去6个月内有≥1次发作。询问患者有关其发作缓解策略的情况,包括增加秋水仙碱剂量、急诊入院、休息、腹部热敷、饮食改变、按需使用阿那白滞素、草药、对乙酰氨基酚、非甾体抗炎药(NSAIDs)以及使用皮质类固醇。在258例患者(98名男性,160名女性;中位年龄35岁,病程18年)中,93%的患者使用了缓解策略。最常见的首选策略是急诊入院(19.8%),其次是增加秋水仙碱剂量(18.2%)、休息(14.3%)、饮食改变(4.3%)、NSAIDs(7%)、对乙酰氨基酚(6.6%)、按需使用阿那白滞素(6.2%)、草药(6.2%)以及皮质类固醇(5%)。C反应蛋白(CRP)水平正常、上次发作持续时间较短、较低的FMF国际严重程度评分(ISSF)、无工作以及非M694V突变与增加秋水仙碱剂量有关(p<0.05)。M694V阳性、有工作、CRP持续升高、主要为浆膜炎和肌肉骨骼发作、教育水平≤8年、上次发作持续时间较长、ISSF较高、当前秋水仙碱耐药和不依从与急诊入院有关(p<0.05)。大多数FMF患者采用策略来缓解发作,主要是急诊入院和增加秋水仙碱剂量。这些受到基因突变、发作类型、工作状态、教育程度以及CRP水平的影响。

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