Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ 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 +... Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth