Right ventricular function in systemic autoimmune diseases

Abstract

Systemic autoimmune diseases (SAD) can be defined as inflammatory diseases with an immune mechanism, of unknown etiology, involving at least 2 organs or systems. This category includes collagen-vascular diseases, vasculitides, granulomatous diseases, while excluding systemic inflammatory diseases with known causes. Cardiovascular changes are among the most frequent causes of morbi-mortality in patients with SAD by many mechanisms, including sustained systemic inflammation. Chronic right ventricular (RV) involvement in SAD may be due to long-term left ventricular (LV) decompensation, inflammation and myocardial fibrosis generated by the disease itself, pulmonary parenchymal changes and/or pulmonary hypertension (PAH), chronic repetitive pulmonary embolism. These differ in type, prevalence, intensity between the various forms of SAD. Systemic sclerosis (SSc) is the most frequent disease that causes lung and cardiac involvement. SS generates PAH by multiple mechanisms mainly in the cutaneous form, although it induces myocardial perfusion defects especially in the diffuse form. The RV involvement occurs earlier in SSc than in other forms of PAH and the prognosis is more severe. However, the new cardiac imaging techniques prove the occurrence of the systolic and diastolic dysfunction in right and left ventricles in cases without established PAH and clinical heart involvement. Systemic erythematosus lupus (SLE), mixed connective-tissue disease (MCTD), Sjogren syndrome (SjS), rheumatoid arthritis (RA) determine less often PAH and RV involvement. There are also other mechanisms for the cardiac involvement in SAD, like coronaritis, accelerated atherosclerosis or pericarditis.