Rheumatoid Arthritis

RA, characterized by a symmetric, additive, destructive synovitis, occurs in 1% of most populations. The most frequent cardiac manifestations in patients with RA are pericarditis and valvular heart disease (Fig. 62-1). These features are more common in patients with nodular seropositive RA than in RA patients without extra-articular pathology. With routine screening echocardiograms, pericardial thickening with or without a pericardial effusion may be seen in up to 60% of patients, though clinically evident in less than 5% (Tables 62-1 and 62-2). Pericardial fluid due to RA involvement is exudative, serosanguineous, or hemorrhagic with high acidity. Adhesions and loculations are common, often making pericardiocentesis ineffective. A significant proportion of patients with clinical pericarditis have constriction or tamponade with a grave prognosis. These patients, under some circumstances, may benefit from surgical pericardiectomy.

Figure 62-1 Extra-articular manifestations in rheumatoid arthritis.

Table 62-1 Clinical Cardiac Manifestations in Rheumatologic Disorders

Table 62-2 Prevalence of Cardiac Involvement in Rheumatologic Disorders

Despite frequent occurrence (up to 70%), valvular lesions are rarely symptomatic in RA. Pathologically, endocardial lesions can be caused by fibrosis, nonspecific inflammation, or rarely, rheumatoid granulomas. Aortic or mitral insufficiency and aortic root dilation are the most common manifestations. When due to inflammation these lesions may progress rapidly and require surgical intervention. Myocarditis is rarely clinically evident but can be associated with arrhythmias. Vasculitis of coronary vessels has been described, although the clinical significance is unknown. Recently, serum levels of antibodies directed against cyclic citrullinated peptide (anti-CCP) have been detected in the sera of RA patients earlier than rheumatoid factor (RF). It has been suggested that anti-CCP may define a subset of individuals at increased risk for destructive arthritis. Strong gene-environment interactions also exist between cigarette smoking and homozygosity for HLA DRB1 shared-epitope (SE) alleles DRB1*04 or DRB1*01. Heterozygous individuals who carry at least one copy of the SE and are exposed to cigarette smoking also have a markedly increased risk of anti-CCP-positive RA. Increased cardiovascular risk occurs in RA independent of traditional risk factors and has been attributed to ongoing inflammation. More aggressive management of inflammation in RA as well as traditional risk factors may lead to marked improvements in outcomes for patients with RA.

Systemic Lupus Erythematosus

SLE (also called lupus) is a multisystem autoimmune disorder characterized by the production of autoantibodies and a striking female predominance in the reproductive years (10 : 1 female-to-male). Autoantibodies and immunocomplexes with complement activation are thought to be the major factors in cardiovascular injury. In SLE, as in RA, the pericardium and endocardium are most commonly involved. Serositis in SLE is often associated with disease flares. Pericarditis is clinically evident in up to 20% of patients, with a prevalence by echocardiography and in autopsy series as high as 60% in individuals with SLE. Tamponade occurs in 1% to 2% of patients; constriction is even less common. Analysis of pericardial fluid is similar to that of RA, with high acidity and increased polymorphonuclear cells.

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