Deniz Rabia, Mandacı Aybüke, Gerdan Ilayda, Özgür Duygu Sevinç, Karaalioğlu Bilgin, Akkuzu Gamze, Yıldırım Fatih, Bes Cemal
Department of Rheumatology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey.
Department of Internal Medicine, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey.
Mediterr J Rheumatol. 2023 Jul 30;34(4):550-554. doi: 10.31138/mjr.300723.aco. eCollection 2023 Dec.
Protracted febrile myalgia syndrome (PFMS) is a rare form of familial Mediterranean fever (FMF) characterised by prolonged myalgia. The duration of PFMS is much longer than a typical 2-5-day attack familial Mediterranean fever and lasts for 2-6 weeks until they treated with corticosteroids. Colchicine is not effective for control of PFMS's attacks. The attacks typically resolve with corticosteroid and/or IL-1 receptor blockers. Herein, we present a young adult without typical familial Mediterranean fever clinical features but presenting with atypical course and severe asymmetric muscle strength loss. Thigh magnetic resonance imaging confirmed inflammation and oedema and muscle biopsy showed no pathological findings. Electromyography revealed myopathic findings during attack-period, despite normal results in attack-free study. The patient was treated successfully with anakinra and remarkable rapid recovery in both muscular findings and acute phase reactants were observed. PFMS should be considered even in the absence of apparent FMF attack pattern and in the presence of unexpected severe muscle weakness, especially in areas endemic for FMF and long-lasting myalgia attacks.
持续性发热性肌痛综合征（PFMS）是家族性地中海热（FMF）的一种罕见形式，其特征为持续性肌痛。PFMS的病程比典型的2至5天发作的家族性地中海热长得多，可持续2至6周，直到使用皮质类固醇进行治疗。秋水仙碱对控制PFMS的发作无效。发作通常通过皮质类固醇和/或IL-1受体阻滞剂得到缓解。在此，我们报告一名年轻成人，其没有典型的家族性地中海热临床特征，但呈现非典型病程和严重的不对称肌肉力量丧失。大腿磁共振成像证实存在炎症和水肿，肌肉活检未发现病理结果。肌电图显示发作期有肌病表现，尽管在无发作期检查结果正常。该患者使用阿那白滞素治疗成功，肌肉表现和急性期反应物均显著快速恢复。即使没有明显的FMF发作模式且存在意外的严重肌肉无力，尤其是在FMF流行地区且有持久的肌痛发作时，也应考虑PFMS。
