Lipoprotein lipase (LPL) is a member of the lipase family, and LPL is known to hydrolyze triglyceride molecules found in lipoprotein particles. Understanding the pathogenesis of atherosclerosis in autoimmune diseases, particularly systemic lupus erythematosus (SLE), is vital to reduce morbidity and mortality of this disease. It is now recognized that SLE has a particular pattern of dyslipoproteinemia characterized by low HDL levels and increased triglycerides, which is aggravated by flare. Antilipoprotein lipase (anti-LPL) antibodies have been recently described in rheumatic diseases, mainly in SLE and systemic sclerosis (SSc). Several studies have reported a close link between antibodies, inflammation and lipoprotein levels and an increased risk of cardiovascular damage in this particular group of patients. These studies have emphasized the importance of rigorous clinical control of disease activity and prevention of cardiovascular risk factors. This review summarized the studies that have discussed the presence of anti-LPL in rheumatic diseases and other conditions and analyzed the importance of this antibody in the complex atherosclerotic process in autoimmune diseases.