Department of Pediatric Rheumatology, Faculty of Medicine, Gazi University, Ankara, Turkey.
Department of Pediatric Rheumatology and Nephrology, Faculty of Medicine, Gazi University, Ankara, Turkey.
Rheumatol Int. 2019 Jan;39(1):147-152. doi: 10.1007/s00296-018-4163-5. Epub 2018 Oct 6.
Protracted febrile myalgia syndrome (PFMS) in familial Mediterranean fever (FMF) patients is a vasculitic condition characterized by severe myalgia, fever, abdominal pain, diarrhea, and arthralgia/arthritis episodes lasting 4-6 weeks. Symptoms typically resolve with corticosteroid treatment. However, in recent years, corticosteroid-resistant PFMS patients have been reported. We herein report five pediatric FMF patients complicated with PFMS. In addition, demographic findings, Mediterranean fever (MEFV) gene analysis, symptoms at disease onset, time interval between the diagnoses of FMF and PFMS, co-existent diseases, and treatment responses were evaluated. Resolution of all PFMS symptoms was accepted as complete response, while decreased symptoms without full recovery as partial response. We searched PubMed using the keywords 'protracted febrile myalgia' and 'anakinra', and reviewed the literature. There were three male and two female patients. Median age at the diagnosis of FMF was 6 (3-10) years. The time from diagnosis of FMF to the development of PFMS was changed from 0 to 8 (median: 2) years. All of the patients, except one, had homozygous M694V mutation. All patients were treated with corticosteroids and non-steroidal anti-inflammatory drugs (NSAIDs) first. Two out of five patients were exhibited partial response, while others exhibited complete response. Patients with partial response to the conventional therapies were treated with anakinra, and achieved a great response after the first dose. Anti-interleukin-1 (IL-1) therapy may be a beneficial and a reasonable treatment option, when there is insufficient response to NSAID and corticosteroid therapies in pediatric PFMS patients.
家族性地中海热（FMF）患者的迁延性发热性肌痛综合征（PFMS）是一种血管炎性疾病，其特征为严重肌痛、发热、腹痛、腹泻和关节痛/关节炎，持续 4-6 周。症状通常通过皮质类固醇治疗缓解。然而，近年来，已报道有皮质类固醇耐药的 PFMS 患者。我们在此报告 5 例儿科 FMF 合并 PFMS 的患者。此外，评估了人口统计学特征、地中海热（MEFV）基因分析、疾病发作时的症状、FMF 和 PFMS 诊断之间的时间间隔、共存疾病和治疗反应。所有 PFMS 症状的缓解被认为是完全缓解，而症状减轻但未完全恢复为部分缓解。我们使用关键词“protracted febrile myalgia”和“anakinra”在 PubMed 上进行了检索，并复习了文献。有 3 例男性和 2 例女性患者。FMF 诊断时的中位年龄为 6（3-10）岁。从 FMF 诊断到 PFMS 发展的时间从 0 到 8（中位数：2）年不等。除 1 例外，所有患者均携带纯合 M694V 突变。所有患者均首先接受皮质类固醇和非甾体抗炎药（NSAIDs）治疗。5 例患者中有 2 例表现为部分缓解，其余表现为完全缓解。对常规治疗有部分反应的患者接受了 anakinra 治疗，首次给药后获得了很好的反应。在儿科 PFMS 患者对 NSAID 和皮质类固醇治疗反应不足时，抗白细胞介素-1（IL-1）治疗可能是一种有益且合理的治疗选择。
