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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Raynaud’s phenomenon, sclerodactyly

  • Myopathy with high titers of ribonucleoprotein antibodies

  • Pericarditis, pulmonary hypertension

GENERAL CONSIDERATIONS

  • Patients with mixed connective tissue disease have clinical features that resemble other connective tissue diseases

  • Characteristically, affected patients have high titers of antibodies to ribonucleoprotein and speckled antinuclear antibodies

  • Rheumatoid agglutinins occur in about 50%

  • Predominantly, females of all ages are affected

  • Cardiac involvement is infrequent and usually related to pericarditis or pulmonary hypertension

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Chest pain characteristic of pericarditis is common

  • Symptoms of heart failure

    • – Fatigue

    • – Dyspnea

    • – Edema

PHYSICAL EXAM FINDINGS

  • Pulmonary hypertension

    • – Right ventricular (RV) lift

    • – Loud P2

    • – Edema

  • Pericarditis

    • – Friction rub

    • – Elevated jugular venous pressure (JVP)

DIFFERENTIAL DIAGNOSIS

  • Other connective tissue diseases

  • Other causes of cardiac disease

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • Antinuclear antibody titer, ribonucleoprotein level, rheumatoid factor

  • Brain natriuretic peptide

ELECTROCARDIOGRAPHY

  • RV and right atrial hypertrophy

  • Diffuse ST elevation of pericarditis

  • Supraventricular arrhythmias

IMAGING STUDIES

  • Echocardiography:

    • – Pericardial effusion

    • – Right heart chamber enlargement

    • – Verrucous mitral valve thickening

  • Doppler echocardiography:

    • – Elevated pulmonary pressure estimates

    • – Mitral regurgitation

  • Chest x-ray: enlarged cardiac silhouette

DIAGNOSTIC PROCEDURES

  • Cardiac catheterization: may be required in some cases to evaluate possible coronary artery disease or cardiac tamponade

TREATMENT

CARDIOLOGY REFERRAL

  • Suspected cardiac disease

HOSPITALIZATION CRITERIA

  • Heart failure

  • Pericarditis

  • Rapid atrial fibrillation

MEDICATIONS

  • Specific anti-inflammatory pharmacotherapy

  • Appropriate therapy for pulmonary hypertension

  • Steroids for pericarditis, eg, prednisone 5–50 mg PO daily

THERAPEUTIC PROCEDURES

  • Pericardiocentesis if tamponade

MONITORING

  • ECG in hospital as appropriate

  • Blood pressure and JVP with pericarditis

  • Pulmonary artery pressures during drug treatment of pulmonary hypertension

DIET AND ACTIVITY

  • Low-sodium diet

  • Restricted activity if heart failure

ONGOING MANAGEMENT

HOSPITAL DISCHARGE CRITERIA

  • Resolution of problem

FOLLOW-UP

  • Cardiology follow-up as appropriate to problem

COMPLICATIONS

  • Atrial and ventricular arrhythmias

  • Myocarditis

  • Cardiac conduction disturbances

PROGNOSIS

  • Mortality is 13% at 10 years

RESOURCES

PRACTICE GUIDELINES

  • Although like other connective tissue diseases, any structure in the heart can become diseased, the usual clinical manifestations are pericarditis and pulmonary hypertension

  • Rarely ...

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