Jaccoud arthropathy (JA) was initially described in patients with rheumatic fever and later in several other rheumatologic conditions, particularly systemic lupus erythematosus (SLE). In patients with the latter disorder, a prevalence of about 5% has been observed. We conducted the current study to describe a series of patients with SLE with JA, followed at the Hospital Santa Izabel, Salvador, Brazil, during the year 2006. We reviewed the literature on JA, with emphasis on the histologic, clinical, radiologic, and therapeutic aspects of the condition. Twenty-one patients with JA were identified, corresponding to a prevalence of 3.47% in the population of 606 patients with the diagnosis of SLE attended in our service. Twenty patients were women, and the mean age was 40.2 +/- 8.8 years (range, 24-55 yr). The most frequently found joint deformities were swan neck and thumb subluxation, both identified in 14 patients. Ulnar deviation was seen in 8, boutonniere deformity in 3, and hallux valgus in 2 patients. We found no difference in the clinical or laboratory features in SLE patients with or without JA. The patients with JA presented a trend toward a lower quality of life compared with the patients with SLE without JA, but without statistical significance.