Primary and Secondary Raynaud: A Scientific Statement From the American Heart Association.

Abstract

Raynaud phenomenon (RP), a vascular disorder affecting the small arteries and arterioles of the extremities, is characterized by episodic vasospastic attacks. In primary RP (PRP), these episodes cause changes in skin color, ranging from white (pallor) to blue (cyanosis) due to reduced tissue perfusion, followed by red (hyperemia) upon reperfusion, often triggered by environmental factors or emotional stress. RP is classified into 2 major types: PRP and secondary RP (SRP). PRP and SRP share similar symptoms, but differ in pathogenesis, severity, duration, and potential complications. PRP usually affects women in the second decade of life and typically follows a benign course. SRP usually occurs later in life, secondary to an underlying systemic condition. Unlike PRP, SRP may result in digit ischemia or tissue loss in severe cases. There is a scarcity of clinical trials evaluating treatment options for patients with RP. Management involves both lifestyle modifications and pharmacologic therapies. Although much remains unknown about the pathophysiology, management, and quality of life in RP, emerging evidence suggests that advanced imaging modalities may facilitate earlier detection of SRP. Treating the underlying disease is essential to improving vascular symptoms and preventing complications in SRP. This scientific statement reviews current evidence on quality of life measures for individuals with RP and on medical management, botulinum toxin therapy, and surgical intervention for severe cases.