What are helpful ways to elicit a history of RP?

RP is an exaggerated response of the digits to cold temperatures and is characterized by distinct color changes of the skin of the digits. It is thought to be due to an abnormal vascular response, causing vasoconstriction of the digital arteries and cutaneous arterioles. An attack of RP classically manifests as a triphasic color change, white to blue to red. RP is considered primary if not associated with an underlying disease (i.e., many young, thin females who are long-distance runners have primary RP; this is usually benign) and secondary RP if it is associated with a connective tissue disease.

There is no standardized way to elicit a history of true RP. The author prefers these screening questions:

What skin changes are seen in patients with SSc?

What are possible causes of this patient’s dyspnea?

Dyspnea in a patient with SSc should always prompt evaluation. Pulmonary involvement is seen in more than 70% of patients with SSc; the most common pulmonary manifestations include interstitial lung disease (ILD) and pulmonary vascular disease, leading to pulmonary arterial hypertension (PAH). PAH is amenable to medical management; this should be assessed for initially with a transthoracic echocardiogram (TTE). However, echocardiograms give both false-negative and false-positive results; right heart catheterization is essential to confirm the diagnosis and exclude other disease processes. Often severe and progressive, PAH can lead to cor pulmonale and right-sided heart failure.