Objective: To characterize cognitive function in patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) in comparison with other chronic conditions, and to investigate its association with disease activity, and other psychological factors. Methods: Cross-sectional study including patients with AAV, rheumatoid arthritis (RA) (n = 30), and chronic kidney disease (CKD) (n = 29). Patients underwent a standardized neuropsychological battery (NEUROPSI). Sleep quality, fatigue, depression, and anxiety levels were assessed. Results: A total of 60 patients with AAV were included, median age of 54 years, and disease duration of 5.6 years. Prevalence of cognitive dysfunction (CD) in AAV patients was similar to RA and CKD (35%, 40%, and 39.3%, respectively, p = .88). When AAV patients with (n = 21) and without (n = 39) CD were compared, significantly more patients with CD had high disease activity (67% vs. 31%, p = .009). Abnormal performance was more frequent in the executive functions in the three groups (45% AAV, 51.7% RA, and 50% CKD), followed by language (25%, 13.8%, and 25%, respectively). Verbal and visual attentional tests were more frequently impaired in patients from the CKD group (p = .021), and psychomotor functions were more frequently affected in AAV patients (p < .05). Hospital Anxiety and Depression Scale (HADS) total score (especially anxiety) was higher in patients with memory impairment than in those with normal memory function (M = 6.79, SD = 4.53 vs. M = 4.5, SD = 3.6, p < .01). Neither Sleep Quality Index nor fatigue scale scores differed between those cognitively impaired and not impaired. Conclusions: No statistically significant differences were found in the frequency of CD among the three clinical populations. (JINS, 2019, 25, 595–602)