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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Sclerotic skin, esophageal dysfunction, Raynaud’s phenomenon

  • Diastolic heart failure

  • Functional and structural small-vessel coronary artery disease (CAD)

  • Multisegmental myocardial perfusion abnormalities in the absence of epicardial CAD

  • Pulmonary hypertension and cor pulmonale

GENERAL CONSIDERATIONS

  • Scleroderma or progressive systemic sclerosis is characterized by excessive accumulation of connective tissue and fibrosis of the skin, skeletal muscles, joints, blood vessels, kidneys, lung, gastrointestinal tract, and heart

  • Scleroderma occurs in 10–20 per million people per year, is more common in women (3:1), and usually occurs in people between ages 30 and 50 years

  • Ninety percent of patients with scleroderma have Raynaud’s phenomenon, esophageal dysfunction, and sclerotic skin changes in a focal (80%) or a diffuse pattern (20%)

    • – The more common focal type is associated with the CREST syndrome (calcinosis, Raynaud’s, esophageal dysfunction, sclerodactyly, and telangiectasia)

    • – Skin changes are often limited to the face and fingers

    • – The diffuse type more frequently involves the abdominal and thoracic organs

  • The diffuse type has a worse prognosis than the focal type

  • The major causes of mortality are pulmonary hypertension, renal dysfunction, and heart disease

  • The cardiac diseases most commonly associated with scleroderma include coronary artery disease, myocarditis, and pulmonary hypertension

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Chest pain is common but is more often due to pericarditis or esophageal reflux than to myocardial ischemia

  • Dyspnea, orthopnea, and edema

PHYSICAL EXAM FINDINGS

  • Typical skin findings of scleroderma, telangiectasia, subcutaneous calcified nodules

  • Signs of heart failure, such as cardiomegaly, S3, jugular venous distention, pulmonary rales, and edema

  • Pericardial friction rub, pulsus paradoxus, fever

  • Right ventricular lift, loud pulmonic component of S2 with pulmonary hypertension

DIFFERENTIAL DIAGNOSIS

  • Typical coronary artery disease

  • Other causes of myopericarditis and heart failure

  • Other causes of pulmonary hypertension and cor pulmonale

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • Elevated cardiac biomarkers in myocarditis or infarction

  • Exudative pericardial effusion

ELECTROCARDIOGRAPHY

  • Septal Q waves usually due to fibrosis rather than infarction

  • Signs of acute pericarditis

  • Conduction abnormalities

  • Nonspecific ST-T–wave changes

  • Right ventricular hypertrophy

IMAGING STUDIES

  • Chest x-ray: enlarged cardiac silhouette

  • Echocardiography may show the following common findings:

    • – Diffuse left ventricular dysfunction or segmental wall motion abnormalities

    • – Left ventricular hypertrophy

    • – Left atrial enlargement

    • – Doppler evidence of diastolic dysfunction

    • – Nonspecific valve thickening and regurgitation

    • – Also seen are pericardial effusion, signs of tamponade, and right ventricular and atrial hypertrophy

  • Rest or exercise radionuclide myocardial perfusion imaging may show multisegmental perfusion defects

  • CT and cardiac MRI are useful for evaluating pericardial thickness

DIAGNOSTIC PROCEDURES

  • Myocardial biopsy may be useful to diagnose scleroderma involvement in ...

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