Inflammatory and thrombotic valvulopathies in autoimmune disease

Abstract

Rheumatologic diseases are characterised by loss of immune tolerance, resulting in systemic inflammation. Inflammation and scarring of the endocardium, which lines the inner surface of the heart chambers and valves, can result in valvular thickening and dysfunction. Estimates of prevalence vary depending on the sensitivity of the screening methodology used and range from 30%–50% in systemic lupus and rheumatoid arthritis to 10%–30% in ankylosing spondylitis. Progression of valve disease is a slow process but can result in haemodynamically significant complications. Thromboembolic complications such as cerebrovascular occlusions pose a serious risk of morbidity. The presence of antiphospholipid antibodies increases the risk of valvular disease and thrombotic complications. Anticoagulation is recommended in the presence of antiphospholipid antibodies, but the guidance on the role of immunosuppressive therapy to treat valvular disease is lacking. Surgical valve therapy may be considered in severe disease, but there is increased risk in patients with an autoimmune disease which includes a higher risk of infection, thromboembolic and bleeding complications, as well as cardiovascular events in the setting of premature atherosclerotic heart disease. Therefore, management should be provided in a multidisciplinary team that includes a rheumatologist, a cardiologist and a cardiothoracic surgeon; medical therapy should be optimised before considering a high-risk valve surgery.

Cardiovascular risk in axial spondyloarthritis—a systematic review

“…Aortic valve insufficiency is one of the most important cardiovascular involvements occurring in 18% of patients [ 50 ]. It has been proposed to be a result of chronic aortitis which involves the aortic root and leads to a dilatation and insufficiency [ 51 , 52 ].…”

Antiphospholipid Antibodies Are Major Risk Factors for Non-Thrombotic Cardiac Complications in Systemic Lupus Erythematosus

“…The pathomechanism of valvulopathies is not yet fully understood. Based on the histological examination of the autopsy samples, mononuclear cell infiltration, endothelial cell proliferation, platelet activation and mycrothrombus formation can be observed [ 10 ]. It has been suggested that APA positivity increases the risk of valve thrombi formation, which may explain the association of APAs with valvular lesions [ 21 ].…”

“…Based on our data, we found the strongest association between the presence of anticardiolipin IgG antibodies and non-thrombotic cardiac manifestations. Based on the results of a recently published meta-analysis, patients with anticardiolipin IgG antibodies can benefit the most from echocardiography screening tests [ 10 ]. At the same time, the incidence of ischemic heart disease and cardiomyopathy was significantly higher in patients with valvulopathy, which draws attention to the fact that non-thrombotic cardiac manifestations can occur not only independently but also in combination with each other.…”

“…However, the greatest risk of thrombosis is the triple antiphospholipid antibody positivity [ 8 , 9 ]. It is known that antiphospholipid antibodies affect the development of cardiac manifestations, but the exact pathomechanism is still not fully understood [ 10 ]. It is also known that antiphospholipid antibodies contribute not only to the development of thrombotic events, but also to accelerated atherosclerosis [ 11 ].…”

Cardiovascular disease in spondyloarthritis: a narrative review of risk factors and the effect of treatments

“…Patients with axSpA have a 4.5-fold higher frequency of aortic valve insufficiency than controls (aHR = 4.5, 95% CI 1.1–13.6) [38]. This may be the result of chronic aortitis leading to aortic root dilatation and valve insufficiency [24,39] and can occur in up to 18% of patients with axSpA [40].…”