Symptoms of Pulmonary Arterial Hypertension (PAH)

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What are the presenting symptoms of Pulmonary Arterial Hypertension (PAH)?

Dyspnea and fatigue are the two most common symptoms of Pulmonary Arterial Hypertension ; yet, both are ubiquitous and unreliable among patients with scleroderma.

Patients often underreport such symptoms, and providers have a difficult time reliably quantifying their severity or progression.

It is important for all scleroderma patients to undergo Pulmonary Arterial Hypertension screening, including patients who do not report dyspnea.

There are few signs of early Pulmonary Arterial Hypertension on physical examination, but several important exam findings suggest advanced Pulmonary Arterial Hypertension (i.e., features of right heart dysfunction including lower extremity edema, the murmur of tricuspid regurgitation, jugular venous distension, hepatomegaly, and right ventricular heave).

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