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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Muscle weakness, characteristic skin lesions

  • Arrhythmias or conduction disturbances

  • Myocarditis, pericarditis, coronary arteritis, and valve disease

GENERAL CONSIDERATIONS

  • Polymyositis/dermatomyositis is a chronic inflammatory myopathy that presents as symmetric proximal muscle weakness and a rash, usually over the extensor surfaces of the hands and the face, neck, and chest

  • The incidence is about 1–5 new cases per million per year in the United States

  • It strikes adults in their fourth to sixth decades

  • African-American women are more often affected

  • Occasionally the condition is associated with other connective tissue diseases

  • Cardiovascular disease is a common cause of mortality

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Palpitation due to ectopic beats

  • Congestive heart failure findings, such as dyspnea on exertion and edema

  • Chest pain due to pericarditis

PHYSICAL EXAM FINDINGS

  • Pericardial rub, elevated jugular venous pressure

  • Pulmonary rales, S3, enlarged apical impulse

  • Right ventricular lift, loud P2

  • Murmur of mitral regurgitation

  • Skin manifestations

DIFFERENTIAL DIAGNOSIS

  • Other connective tissue diseases

  • Other causes of cardiac disease

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • Elevated brain natriuretic peptide or troponins

ELECTROCARDIOGRAPHY

  • ST-T–wave abnormalities in about 50%

  • Fascicular and bundle branch blocks

  • Atrioventricular blocks

  • Premature atrial and ventricular beats

IMAGING STUDIES

  • Chest x-ray:

    • – Cardiomegaly

    • – Pulmonary congestion

  • Echocardiography:

    • – Pericardial effusion

    • – Left ventricular wall motion abnormalities and reduced ejection fraction

    • – Enlarged right heart and Doppler evidence of increased pulmonary pressure

    • – Mitral valve prolapse in about 50%

    • – Hyperkinetic heart syndrome in some

  • Myocardial perfusion imaging with technetium-99m pyrophosphate shows myocardial uptake

  • Cardiac MRI with late gadolinium enhancement of the epicardium and mid-wall of the lateral and septal walls

DIAGNOSTIC PROCEDURES

  • Cardiac catheterization or CT coronary angiography may be required if coronary vasculitis is suspected

TREATMENT

CARDIOLOGY REFERRAL

  • Suspected heart disease

HOSPITALIZATION CRITERIA

  • Pericarditis

  • Heart failure

  • Significant arrhythmias or heart block

MEDICATIONS

  • Specific anti-inflammatory pharmacotherapy

  • Specific cardiac therapy as indicated

  • Pulmonary vasodilators in selected cases

THERAPEUTIC PROCEDURES

  • Percutaneous coronary intervention may be required

  • Pericardiocentesis may be required.

SURGERY

  • Valve, coronary, or pericardial surgery is rare

MONITORING

  • ECG monitoring in hospital as appropriate

DIET AND ACTIVITY

  • Low-sodium diet if heart failure

  • Restricted activity if heart failure or myocarditis

ONGOING MANAGEMENT

HOSPITAL DISCHARGE CRITERIA

  • Resolution ...

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