Cardiorheumatology: cardiac involvement in systemic rheumatic disease

Abstract

Autoimmune rheumatic diseases can affect the cardiac vasculature, valves, myocardium, pericardium, and conduction system, leading to a plethora of cardiovascular manifestations that can remain clinically silent or lead to substantial cardiovascular morbidity and mortality. Although the high risk of cardiovascular pathology in patients with autoimmune inflammatory rheumatological diseases is not owing to atherosclerosis alone, this particular condition contributes substantially to cardiovascular morbidity and mortality-the degree of coronary atherosclerosis observed in patients with rheumatic diseases can be as accelerated, diffuse, and extensive as in patients with diabetes mellitus. The high risk of atherosclerosis is not solely attributable to traditional cardiovascular risk factors: dysfunctional immune responses, a hallmark of patients with rheumatic disorders, are thought to cause chronic tissue-destructive inflammation. Prompt recognition of cardiovascular abnormalities is needed for timely and appropriate management, and aggressive control of traditional risk factors remains imperative in patients with rheumatic diseases. Moreover, therapies directed towards inflammatory process are crucial to reduce cardiovascular disease morbidity and mortality. In this Review, we examine the multiple cardiovascular manifestations in patients with rheumatological disorders, their underlying pathophysiology, and available management strategies, with particular emphasis on the vascular aspects of the emerging field of 'cardiorheumatology'.

Figure 1

Multiple cardiovascular manifestations of rheumatic diseases. Autoimmune systemic diseases can have multiple associated cardiovascular manifestations, which can largely be categorized as being vascular, myocardial, valvular, pericardial, or electrical.

Figure 2

Common mechanisms underlying atherosclerosis and rheumatoid arthritis. Both conditions are associated with upregulation of TNF-α, metalloproteinase expression, upregulation of IL-6, T-cell activation, elevated C-reactive protein level, increased expression of adhesion molecules and endothelin, and activation of macrophages. Autoantibodies to oxLDL participate in development of both atherosclerosis and rheumatoid arthritis. Abbreviations: IL-6, interleukin-6; oxLDL, oxidized low-density lipoprotein; TNF-α, tumour necrosis factor-α; VCAM-1, vascular cell adhesion protein 1. Reprinted with modifications with permission from Elsevier © Am. J. Med. 121 (Suppl. 1), Libby, P. Role of inflammation in atherosclerosis associated with rheumatoid arthritis, S21–S31 (2008).

Figure 3

Cumulative incidence of cardiovascular manifestations in patients with or without RA. Cumulative incidence of silent MI, sudden cardiac death, and angina in cohorts with or without RA, after adjusting for the competing risk of death from other causes. Abbreviations: MI, myocardial infarction; RA, rheumatoid arthritis. Permission obtained from Wiley © Maradit-Kremers, H. et al. Increased unrecognized coronary heart disease and sudden deaths in rheumatoid arthritis: a population-based cohort study. Arthritis Rheum. 52, 402–411 (2005).

Figure 4

Cumulative incidence of congestive heart failure in patients with or without RA. Patients with T2DM and patients with RA have a similar cumulative incidence of congestive heart failure, after adjusting for the risk of death from other causes, compared with individuals without RA or diabetes. Abbreviations: RA, rheumatoid arthritis; T2DM, type 2 diabetes mellitus. Permission obtained from Wiley © Peters, M. J. et al. Does rheumatoid arthritis equal diabetes mellitus as an independent risk factor for cardiovascular disease? A prospective study. Arthritis Rheum. 61, 1571–1579 (2009).