Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.
Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Matsumoto, and Department of Clinical Research, National Hospital Organization Matsumoto Medical Center, Matsumoto, Japan.
Clin Exp Rheumatol. 2020 Sep-Oct;38 Suppl 127(5):76-79. Epub 2020 Nov 4.
We occasionally encounter patients with familial Mediterranean fever (FMF) whose attacks are triggered by specific factors; however, information regarding these factors is limited. Our purpose was to identify the factors that trigger febrile attacks in Japanese patients with FMF.
Our retrospective study included 372 patients (229 women, 143 men) with FMF, who were diagnosed between April 2007 and June 2018. We retrospectively investigated clinical features, genetic variants, and the factors that the patients perceived to have triggered their attacks. Patients completed a questionnaire that included the following triggering factors, anxiety, psychological stress, tiredness, excitement, environmental change, and menstruation.
Of 372 patients, 180 (49.4%) reported some triggering factors. Psychological stress and tiredness were commonly reported factors regardless of sex; however, menstruation (39.7%, n=91) was the most commonly reported triggering factor in female patients with FMF. Menstrual-related patients had a younger age of onset and diagnosis, a higher frequency of peritonitis, and a higher rate of patients with endometriosis compared with the non-menstrual-related patients.
Gaining an understanding of these triggering factors could help to reduce attacks and educate the patients. Clinicians may need to consider FMF for patients who have fever and serositis that occurs with every menstrual period.
我们偶尔会遇到一些家族性地中海热（FMF）患者，他们的发作是由特定因素引发的；然而，关于这些因素的信息有限。我们的目的是确定触发日本 FMF 患者发热发作的因素。
我们的回顾性研究包括 372 名（229 名女性，143 名男性）FMF 患者，他们于 2007 年 4 月至 2018 年 6 月期间被诊断为 FMF。我们回顾性地调查了临床特征、遗传变异以及患者认为触发其发作的因素。患者完成了一份问卷，其中包括以下触发因素：焦虑、心理压力、疲劳、兴奋、环境变化和月经。
在 372 名患者中，有 180 名（49.4%）报告了一些触发因素。心理压力和疲劳是无论性别都常见的因素；然而，月经（39.7%，n=91）是 FMF 女性患者最常见的触发因素。与非月经相关的患者相比，月经相关的患者发病年龄和诊断年龄更小，腹膜炎的发生频率更高，子宫内膜异位症的患者比例更高。
了解这些触发因素有助于减少发作并对患者进行教育。对于每个月经周期都伴有发热和浆膜炎的患者，临床医生可能需要考虑 FMF。
