Systemic lupus erythematosus (SLE), primary antiphospholipid syndrome (PAPS), rheumatoid arthritis (RA), ankylosing spondylitis (AKS), and scleroderma are immune mediated inflammatory diseases that manifest predominantly with symptoms and signs of musculoskeletal and mucocutaneous inflammation. However, they frequently cause cardiovascular disease, which is associated with substantial morbidity and mortality, and the clinical detection of cardiovascular disease is significantly lower than that of cardiac imaging or post-mortem series. Therefore, an increased awareness and understanding of these diseases may lead to an increase in recognition, treatment, and prevention of the associated cardiovascular involvement. This article will review valvular and coronary heart diseases as the most clinically relevant conditions associated with systemic inflammatory diseases.

SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

Valvular heart disease

Valvular heart disease (VHD) is the most frequent type of heart disease associated with SLE; is associated with significant morbidity; and is a leading cause of death in SLE patients.1 2

Pathology

In post-mortem series the frequency of VHD ranges from 13–100%. Libman–Sacks vegetations are cauliflower-like or flat, red, spreading masses of >2 mm in diameter, more commonly located on leaflets closure lines.1 Active vegetations have central fibrinoid degeneration with fibroblastic proliferation and fibrosis, surrounded by mononuclear and polymorphonuclear cellular infiltration, small haemorrhages and peripherally by thrombus. Healed vegetations have central fibrosis, minimal or no inflammation, and none or endothelialised thrombus. Active, healed and mixed vegetations can occur in a valve. Most vegetations have associated leaflet thickening.

Pathophysiology

Subendothelial deposition of immunoglobulins and complement lead to an increased expression of α3β1 integrin on endothelial cells, increased amounts of collagen IV, laminin and fibronectin, proliferation of blood vessels, inflammation, thrombosis and finally fibrosis. Antiphospholipid antibodies (aPL) may cause valve injury by activation of endothelial cells and upregulation of expression of adhesion molecules, secretion of cytokines, and decreased production of prostacyclins; increased uptake of oxidised low density lipoprotein (LDL) cholesterol by macrophages leading …