Diagnosis and management of scleroderma peripheral vascular disease

Abstract

The manifestations of peripheral vascular disease in patients who have systemic sclerosis (SSc) range from episodic Raynaud's phenomenon to irreversible tissue injury with ulceration and gangrene. Structural and functional changes may occur in the microvessels, digital arteries, and sometimes more proximal vessels. This article discusses the assessment of patients who have Raynaud's phenomenon in whom an underlying scleroderma-spectrum disorder is suspected and patients who have SSc with critical digital ischemia/ulceration. Different imaging techniques, including capillaroscopy and angiography, complement the history and examination, and developments in vascular imaging should facilitate future studies of pathogenesis and treatment response. New vasoactive treatments are currently being researched and older treatments revisited; therefore new approaches to therapy will likely be developed over the next 5 to 10 years.