Why is baseline cardiopulmonary testing important in patients with scleroderma

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Why is baseline cardiopulmonary testing important in patients with scleroderma and what testing is recommended?

The leading causes of death in SSc are lung and heart disease. All patients with SSc require initial cardiopulmonary assessments and longitudinal surveillance for development of lung or heart involvement, as data suggests better outcomes in patients with earlier stage disease. Baseline testing includes:

• Thoracic high-resolution computed tomography (HRCT) scan. Chest X-ray is inadequate.

• Complete pulmonary function tests (PFTs; lung volumes, spirometry, and diffusing capacity for carbon monoxide [DLco])

• Echocardiography

• Six-minute walk testing to assess for oxygen desaturation (best to use a forehead probe as finger pulse-oximetry can be unreliable due to Raynaud’s phenomenon)

• Electrocardiogram (EKG) to screen for conduction system abnormalities. Ambulatory EKG monitoring (Holter monitor) has been shown to be more sensitive than a resting 12-lead EKG, and in one study, it was helpful in identifying patients at risk for sudden cardiac death (e.g., population whom may benefit from implantable cardiac defibrillator placement).

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