Bolla Eleana, Karamanakos Anastasios, Fragoulis George E, Iliopoulos Alexios
Department of Rheumatology, 417 Army Share Fund Hospital (NIMTS), 10-12 Monis Petraki Str, 11521, Athens, Greece.
Department of Rheumatology, Evangelismos General Hospital, Athens, Greece.
Rheumatol Int. 2024 Dec;44(12):3119-3125. doi: 10.1007/s00296-024-05736-9. Epub 2024 Oct 22.
Patients with familial mediterranean fever (FMF) often present with musculoskeletal involvement typical of spondyloarthropathy (SpA) or ankylosing spondylitis (AS), posing a diagnostic challenge for medical practitioners and leading to the description of the FMF-related SpA/AS clinical spectrum. Currently, the available data focuses on SpA diagnosis in patients with known FMF, while the contrary is rarely reported in the medical literature. We describe an unusual case of concomitant FMF diagnosis in a patient with an eight-year long history of typical, human leukocyte antigen-B27 positive AS on adalimumab treatment, who presented with recurrent febrile attacks and abdominal pain. The laboratory work-up revealed high titres of serum amyloid A while genetic testing was positive for the pathogenic M694V heterozygous variant in the MEFV gene. The patient was promptly treated with colchicine, showing complete remission of clinical symptoms and normalisation of inflammatory markers to date. We also performed a review of the available literature elaborating on the interrelationship of AS and FMF in terms of pathogenesis and clinical characteristics. Our case highlights the need for reporting of similar cases and further explores the association of AS and FMF as distinct clinical entities or as constituents of the same disease continuum model.
家族性地中海热(FMF)患者常表现出脊柱关节病(SpA)或强直性脊柱炎(AS)典型的肌肉骨骼受累情况,这给医生带来了诊断挑战,并促使人们对FMF相关的SpA/AS临床谱进行描述。目前,现有数据聚焦于已知FMF患者的SpA诊断,而医学文献中很少报道相反的情况。我们描述了一例不寻常的病例,该患者有长达八年典型的、人类白细胞抗原B27阳性AS病史,正在接受阿达木单抗治疗,出现反复发热发作和腹痛,同时被诊断为FMF。实验室检查显示血清淀粉样蛋白A水平升高,而基因检测显示MEFV基因的致病性M694V杂合变异呈阳性。该患者立即接受了秋水仙碱治疗,迄今为止临床症状完全缓解,炎症指标恢复正常。我们还对现有文献进行了综述,阐述了AS和FMF在发病机制和临床特征方面的相互关系。我们的病例强调了报告类似病例的必要性,并进一步探讨了AS和FMF作为不同临床实体或同一疾病连续体模型组成部分之间的关联。