Chapter 47

Cardiovascular Manifestations of Connective Tissue Disorders and the Vasculitides

Nishant R. Shah, MD

1. What is the leading cause of death in rheumatoid arthritis (RA) and what are the most common cardiac manifestations of RA?

    The leading cause of death in RA is ischemic heart disease. Risk factors attributable to RA include chronic proinflammatory and prothrombotic states, endothelial dysfunction, dyslipidemia, insulin resistance, increased oxidative stress, nonsteroidal antiinflammatory drug (NSAID) use, and corticosteroid use. Other cardiac manifestations of RA include increased risk of congestive heart failure (CHF), pericarditis, and conduction block caused by myocardial rheumatoid nodules.

2. What are the cardiovascular manifestations of systemic lupus erythematosus (SLE)?

    The most common cardiac complication of SLE is pericarditis. Clinically evident myocarditis also occurs in 8% to 25% of patients. Libman-Sacks endocarditis is discussed later. Finally, premature atherosclerosis, due to many of the same independent risk factors noted previously for RA, is now recognized as a major cause of morbidity and mortality.

3. What are the cardiovascular consequences of NSAIDs with predominantly cyclooxygenase-2 (COX-2) inhibition?

    COX-2 inhibitors cause a shift toward thrombosis through reduced endothelial production of prostacyclin (a COX-2–mediated anti-thrombotic process) and relative sparing of platelet production of thromboxane A₂ (a COX-1–mediated pro-thrombotic process). For this reason, concurrent low-dose aspirin is recommended for patients on COX-2 inhibitors. Secondly, COX-2 inhibitors increase sodium and water retention, predisposing patients to edema, CHF exacerbations, and hypertension. Finally, COX-2 inhibitors prevent protective COX-2 upregulation in the setting of myocardial ischemia and infarction, leading to larger infarct size and increased risk of myocardial rupture. Of note, NSAIDs with predominantly COX-1 inhibition are associated with increased risk of gastrointestinal (GI) bleeding. For this reason, concurrent proton-pump inhibitor therapy is recommended for patients on COX-1 inhibitors.

4. What is the major cardiovascular concern associated with tumor necrosis factor (TNF)-α antagonists?

    Results from the Anti-TNF Therapy Against Congestive Heart Failure (ATTACH) trial suggest that high-dose TNF-α antagonist therapy actually increases death from any cause and heart failure hospitalization in patients with New York Heart Association (NYHA) class III/IV CHF.

5. What are the clinical manifestations of antiphospholipid antibody syndrome?

    Antiphospholipid antibodies (APLAs) promote intravascular clotting and can be found in primary APLA syndrome or secondary to other conditions, most commonly SLE. Clinical manifestations of APLA syndrome include spontaneous venous and arterial thromboses, strokes and neurologic syndromes, digital and extremity ischemia, livedo reticularis, thrombocytopenia, and recurrent spontaneous abortions. From a cardiac standpoint, acute coronary thromboses and diffuse small-vessel clotting resulting in global myocardial dysfunction have been described. In addition, Libman-Sacks endocarditis, defined by sterile vegetations on the mitral > aortic/tricuspid > pulmonary valves, is thought to arise from organization of thrombi and can cause valvular regurgitation or stenosis requiring surgical correction. Treatment for APLA syndrome is warfarin (goal international normalized ratio [INR] of 2.0-3.0) +/− daily low-dose aspirin, +/− hydroxychloroquine. Drugs associated with drug-induced lupus are given in Table 47-1.

TABLE. 47-1

DRUGS ASSOCIATED WITH DRUG-INDUCED LUPUS

Drugs Definitively Known to Cause Drug-Induced Lupus	Cardiovascular Medications that Probably Cause Drug-Induced Lupus
Procainamide Hydralazine Diltiazem TNF-α antagonists Minocycline Chlorpromazine Quinidine D-Penicillamine Isoniazid Methyldopa Interferon-α	β-Blockers Captopril Hydrochlorothiazide Amiodarone

β-Blockers, Beta-adrenergic blocking agents; TNF, tumor necrosis factor.

6. Describe the characteristic myocardial lesions of scleroderma and systemic sclerosis and their clinical manifestations.

Only gold members can continue reading. Log In or Register to continue

Share this:

• Click to share on Twitter (Opens in new window)
• Click to share on Facebook (Opens in new window)

Related

Related posts:

1. Prosthetic Heart Valves
2. Cardiovascular Physical Examination
3. Endomyocardial Biopsy
4. Pulmonary Hypertension

Stay updated, free articles. Join our Telegram channel

Join

Full access? Get Clinical Tree