Monitoring and Diagnostic Approaches for Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis

Pulmonary arterial hypertension (PAH) is one of the leading causes of death in patients with systemic sclerosis (SSc). Given the high prevalence and poor survival of SSc-PAH, and that aggressive management of mild disease may be associated with better outcomes, screening is critical. Right heart catheterization (RHC) is the gold standard for the definitive diagnosis of PAH, and should be performed in those patients in whom this diagnosis is suspected. Once a diagnosis of PAH is confirmed by RHC, treatment with PAH-specific therapies should be initiated as soon as possible.

Key points

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Pulmonary arterial hypertension (PAH) is one of the leading causes of death in patients with systemic sclerosis (SSc).
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Known risk factors for the development of SSc-PAH are older age, longer disease duration, a low carbon monoxide diffusion in the lung, a low diffusing capacity of carbon monoxide, and high forced vital capacity/diffusing capacity of carbon monoxide.
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Given the high prevalence and poor survival of SSc-PAH, and that aggressive management of mild disease may be associated with better outcomes, screening is critical.
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Right heart catheterization (RHC) is the gold standard for the definitive diagnosis of PAH, and should be performed in those patients in whom this diagnosis is suspected.
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Once a diagnosis of PAH is confirmed by RHC, treatment with PAH-specific therapies should be initiated as soon as possible.