OBJECTIVE: The prevalence of metabolic syndrome (MetS) tends to be high among rheumatic patients, and cardiovascular disease is the leading cause of death in these conditions. We aimed to determine the prevalence of MetS in patients with Takayasu arteritis (TA) and its association with risk factors and adipokine and cytokine levels. METHODS: A cross-sectional study was conducted in 45 consecutive women with TA and 47 healthy controls matched by age and body mass index. RESULTS: The prevalence of MetS (International Diabetes Federation/American Heart Association criteria) was higher in TA compared to controls (33.34 vs 8.51%, p = 0.003). Patients with TA had a higher frequency of hypertension (p < 0.001) and dyslipidemia (p = 0.001) and higher levels of insulin (p = 0.021), homeostasis model assessment index (p = 0.024), apolipoprotein E (p = 0.029), resistin (p = 0.018), and C-reactive protein (CRP, p < 0.001) compared to healthy subjects, with similar levels of adiponectin and plasminogen activator inhibitor-1 (PAI-1; p > 0.05). Further analysis of patients with TA with and without MetS revealed a higher frequency of overweight/obesity (66.66 vs 26.66%, p = 0.022), higher Framingham score ≥ 1 (p = 0.032), and lower adiponectin levels (20.37 ± 21.16 vs 38.64 ± 22.62 µg/ml, p = 0.022) in the patients with MetS. No differences were found regarding disease duration, activity, glucocorticoid use, resistin, and PAI-1 levels in the 2 groups of patients with TA (p > 0.05). Patients with and without MetS showed no differences in cytokine levels [interleukin 12 (IL-12, IL-1a, IL-6) and tumor necrosis factor-α]. IL-6 had a positive Pearson correlation with CRP only in TA patients with MetS (r = 0.57; p = 0.050). CONCLUSION: A high prevalence of MetS was observed in patients with TA and this comorbidity seems to identify a subgroup of overweight/obese patients with high cardiovascular risk without a significant association with disease status. Further longitudinal studies are necessary to observe the effects of controlling this modifiable risk factor in the quality of life and survival of patients with TA.