Batu Ezgi Deniz, Bayindir Yagmur, Sener Seher, Balik Zeynep, Aliyev Emil, Kasap Cuceoglu Muserref, Basaran Ozge, Bilginer Yelda, Ozen Seza
Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Rheumatology (Oxford). 2025 Mar 1;64(3):1341-1346. doi: 10.1093/rheumatology/keae256.
FMF is characterized by febrile polyserositis attacks. Menstruation could be a trigger for attacks. We aimed to analyse the features of adolescent FMF patients with menstruation-associated attacks and propose a management algorithm.
All female FMF patients who had menarche and visited the Pediatric Rheumatology Unit between January and December 2022, were included into this study. Demographics, general characteristics and the features of menstrual cycle and FMF attacks were noted.
A total of 151 female FMF patients were included. Thirty-five (23.2%) had menstruation-associated attacks. Fever and arthritis were less frequent during the menstruation-associated attacks than the attacks not associated with menstruation in these patients (65.7% vs 88.6%, P = 0.01 and 2.9% vs 20%, P = 0.04, respectively). Patients with menstruation-associated FMF attacks were younger at symptom onset and diagnosis (2.5 vs 5 years, P = 0.004 and 4 vs 7 years, P = 0.01; respectively), had a higher rate of dysmenorrhea (74.3% vs 38.8%, P < 0.001, respectively) and higher pre- and post-menarche attack frequency (4 vs 2 and 10 vs 0, respectively; P < 0.001 for both) than patients whose attacks were not associated with menstruation. The interventions for menstruation-associated attacks included initiating colchicine, increasing the dose of colchicine, switching from coated to compressed colchicine tablets or adding anti-interleukin 1 drugs and initiating on-demand non-steroidal anti-inflammatory drugs, on-demand glucocorticoids and on-demand anakinra. On-demand therapies were beneficial in controlling menstruation-associated attacks.
This is the largest cohort of adolescent FMF patients with menstruation-associated attacks. Severe FMF may cause a tendency to this association. On-demand therapies could be preferred in the management.
家族性地中海热（FMF）的特征为发热性多浆膜炎发作。月经可能是发作的诱因。我们旨在分析青春期FMF患者月经相关发作的特征，并提出一种管理方案。
纳入2022年1月至12月间所有初潮且就诊于儿科风湿病科的女性FMF患者。记录人口统计学、一般特征以及月经周期和FMF发作的特征。
共纳入151例女性FMF患者。35例（23.2%）有月经相关发作。在这些患者中，与月经无关的发作相比，月经相关发作时发热和关节炎的发生率更低（分别为65.7%对88.6%，P = 0.01；2.9%对20%，P = 0.04）。月经相关FMF发作的患者症状发作和诊断时年龄更小（分别为2.5岁对5岁，P = 0.004；4岁对7岁，P = 0.01），痛经发生率更高（分别为74.3%对38.8%，P < 0.001），初潮前后发作频率更高（分别为4次对2次和10次对0次；两者P均< 0.001）。月经相关发作的干预措施包括开始使用秋水仙碱、增加秋水仙碱剂量、从肠溶片换为普通片或加用抗白细胞介素1药物，以及按需使用非甾体抗炎药、糖皮质激素和阿那白滞素。按需治疗有助于控制月经相关发作。
这是最大规模的青春期FMF月经相关发作患者队列。严重的FMF可能导致这种关联倾向。管理中可优先选择按需治疗。
