Department of Pediatric Rheumatology, Dr. Sami Ulus Maternity and Child Health and Diseases Training and Research Hospital, Ankara, Turkey.
Department of Pediatric Nephrology and Rheumatology, Dr. Sami Ulus Maternity and Child Health and Diseases Training and Research Hospital, Ankara, Turkey.
Mod Rheumatol. 2023 Aug 25;33(5):1030-1035. doi: 10.1093/mr/roac087.
Familial Mediterranean fever (FMF) is an auto-inflammatory disease that causes recurrent episodes of fever, abdominal pain, chest pain, and arthritis. Although FMF is well known, protracted febrile myalgia syndrome (PFMS) is a clinical condition that is rare and difficult to diagnose than other symptoms of FMF. PFMS causes fever, myalgia, and acute phase reactant elevation that lasts 2-4 weeks if corticosteroid treatment is not given. In some cases, fever may not be seen. The purpose of this report is to share our experience with PFMS patients in our clinic.
This is an observational, retrospective, single-centre study. We evaluated patients who had been diagnosed with PFMS at our paediatric rheumatology clinic.
Protracted febrile myalgia syndrome was observed in 14 patients. Nine of the patients were female. The median age at the time of diagnosis of PFMS was 10 years. Only three patients had previously been diagnosed with FMF. Most of our patients were patients who had no previous complaint of FMF. PFMS attack was seen as the first clinical manifestation of FMF in 11 patients. Two patients who did not respond to steroid treatment improved with anakinra treatment.
PFMS is a rare condition of FMF disease. It may be the first clinical manifestation of FMF disease. Fever may not be seen in all patients. Clinicians should be aware of this situation.
家族性地中海热（FMF）是一种自身炎症性疾病，可导致反复发作的发热、腹痛、胸痛和关节炎。尽管 FMF 广为人知，但迁延性发热性肌痛综合征（PFMS）是一种罕见且难以诊断的临床病症，其症状比 FMF 的其他症状更难诊断。PFMS 可引起发热、肌痛和急性期反应物升高，如果不给予皮质类固醇治疗，这种情况可持续 2-4 周。在某些情况下，可能不会出现发热。本报告旨在分享我们在诊所中治疗 PFMS 患者的经验。
这是一项观察性、回顾性、单中心研究。我们评估了在我们儿科风湿病诊所被诊断为 PFMS 的患者。
在 14 名患者中观察到迁延性发热性肌痛综合征。9 名患者为女性。PFMS 诊断时的中位年龄为 10 岁。仅有 3 名患者先前被诊断为 FMF。我们的大多数患者以前都没有 FMF 的投诉。11 名患者的 PFMS 发作是 FMF 的首发临床表现。2 名对类固醇治疗无反应的患者在接受 anakinra 治疗后病情得到改善。
PFMS 是 FMF 疾病的一种罕见病症。它可能是 FMF 疾病的首发临床表现。并非所有患者都会出现发热。临床医生应注意这种情况。
