The objective of the study was to evaluate the association between heart valve calcification and atherosclerosis and outcome in systemic lupus erythematosus (SLE). One-hundred and seven patients with SLE (mean age 45.9 +/- 14.7 years) were studied by 2D transthoracic echocardiography. Mitral annulus calcification (MAC) was detected in 24 patients (22.6%) and aortic valve calcification (AVC) in 22 (20.1%). Both MAC and AVC were associated with older age (r = 0.2, p = 0.02; r = 0.40, p <or= 0.001, respectively), high SLE damage index (r = 0.3, p = 0.005; r = 0.40, p = 0.001, respectively), diabetes mellitus (r = 0.2, p = 0.05; r = 0.3, p = 0.003, respectively), hyperlipidemia (r = 0.03, p = 0.01; r = 0.03, p = 0.001, respectively), hypertension (r = 0.20, p = 0.07; r = 0.20, p = 0.08, respectively), serum IgA isotype of anticardiolipin antibody (r = 0.03, p = 0.03; r = 0.04, p = 0.02, respectively), increased serum creatinine (r = 0.03, p = 0.0005; r = 0.12, p = 0.02, respectively), and stroke (r = 0.3, p = 0.0008; r = 0.35, p = 0.0002, respectively). In addition, MAC was associated with coronary artery disease (r = 0.2, p = 0.05). Both MAC and AVC were significantly associated with death during the follow-up period (n = 9, 8.6%) (r = 0.20, p = 0.05; r = 0.20, p = 0.03, respectively). On stepwise logistic regression analysis, MAC and AVC are independently associated with hyperlipidemia and antiphospholipid antibodies. In conclusion, MAC and AVC are prevalent among young SLE patients, positively correlate with premature diffuse atherosclerosis, and are a risk factor for subsequent all-cause mortality.