The objective of this study was to compare the clinical and laboratory characteristics of early-onset familial Mediterranean fever patients (EOFPs), adult-onset familial Mediterranean fever (FMF) patients (AOFPs), and late-onset FMF patients (LOFPs). This study included a total of 202 FMF patients aged 18 years and above. Mediterranean fever (MEFV) gene mutations, demographic data, clinical characteristics, medications patients are on, neutrophil to absolute lymphocyte ratio (NLR), and C-reactive protein (CRP) levels obtained during an attack period, and three weeks after the attack were recorded. Based on the age of symptom onset, patients were divided into three groups: <20 years (EOFPs), 20–39 years (AOFPs), and ≥40 years (LOFPs). The most common symptom was abdominal pain, followed by fever. Fever was statistically significantly more common in EOFPs compared to LOFPs (p=0.001). Most patients with the M694V homozygous mutation had a disease onset below 20 years of age, whereas no compound heterozygous mutation was found in LOFPs. The body mass index (BMI) in EOFPs was lower than in AOFPs and LOFPs (p=0.002). In the attack-free group, patients with the M694V homozygous mutation had significantly higher NLRs (median, 2.36 vs. 2.01, p=0.042). LOFPs had a milder form of the disease with less frequent abdominal pain and fever. We would like to advise clinicians that the NLR can be used to detect acute and subacute inflammation, especially in patients with the M694V homozygous mutation among EOFPs

The current report presents two cases with leg ulcers related to Behçet’s disease (BD) resistant to conventional immunosuppressive therapy (CIST) but successfully treated with adalimumab (ADA). BD, which can affect vessels of any size and type, is a systemic vasculitis. In the vascular system, veins are the most predominantly affected blood vessels, with deep vein thrombosis and recurrent superficial vein thrombophlebitis being the most common vascular signs of the disease in the lower extremities. Leg ulcers, commonly associated with vasculitis or deep vein thrombosis, are rare in patients with BD. Conventional immunosuppressive therapy is very critical to prevent relapses and diminish the risk of post-thrombotic syndrome. In patients with BD-associated venous thrombosis (deep vein thrombosis or superficial vein thrombophlebitis) resistant to these treatments, tumour necrosis factor-α inhibitors can be used alone or in combination with traditional disease-modifying antirheumatic drugs. In view of such information, add-on adalimumab treatment was considered appropriate for both patients. Response to this intervention was highly satisfying for the patients at the end of the 6-month treatment. Nonetheless, it warrants further studies directly evaluating the efficacy of tumour necrosis factor-α inhibitors alone in leg ulcers in BD.