An association between acute myocardial infarction (AMI) and systemic lupus erythematosus (SLE) has been suggested. The cause of AMI is presumed to be atherothrombosis. In the present study, the primary objective was to assess incident AMI cases and the secondary objective was to estimate the proportion of myocardial infarction with nonobstructive coronary arteries (MINOCA) in patients with SLE. All Swedish patients with SLE without AMI before 1996 (n = 4192) were followed for 20 years in the national patient registry. For each SLE patient, 10 age- and sex-matched controls without SLE and AMI before 1996 (n = 41 892) were identified. Data from patients and controls with AMI after 1996 were linked with the Swedish coronary angiography and angioplasty register; 549 (13%) and 3352 (8%) first AMIs occurred in patients with SLE and controls, respectively. The incidence of AMI was 9.6 (95% CI: 8.9-10.5) and 4.9 (95% CI: 4.8-5.1) events/1000 person-years in patients with SLE and controls, respectively. The proportion of MINOCA was 10.8% in patients with SLE and 13.8% in controls (P = .261), respectively. In conclusion, the incidence of AMI is increased in a European population of patients with SLE but there is no indication that the proportion of MINOCA is increased in these patients.