Critical Ischemia in Patients with Raynaud’s Phenomenon

Raynaud’s phenomenon (RP) is an episodic phenomenon in which the skin of the extremities undergoes a classical color change of white (the physiological basis for which is ischemia), blue (cyanosis), and red (hyperemia). The condition may be associated with significant discomfort and pain (Block and Sequeira, Lancet 357:2042–2048, 2001; Wigley, N Engl J Med 347:1001–1008, 2002). RP is usually idiopathic (primary RP), but may occur due to a driving etiology (secondary RP). Primary RP does not progress to digital ulceration or critical ischemia. Classification criteria for primary RP have been proposed and are widely used by clinicians (LeRoy and Medsger, Clin Exp Rheumatol 10:485–488, 1992; Maverakis et al., J Autoimmun 48–49:60–65, 2014). There is a wide range of causes of secondary RP, which may (albeit rarely) progress to critical digital ischemia with potential gangrenous progression requiring amputation. The distinction between primary and secondary RP is important as both the prognosis and management may differ significantly. The aim of this chapter is to describe the background, clinical evaluation, and treatment of the patient with RP who then presents with critical digital ischemia. Most of our review applies especially to patients with systemic sclerosis (SSc) because this is where the (limited) evidence base is the strongest.

The first section of the chapter deals with general principles of assessment and treatment, initially in the patient in whom the cause of secondary RP is unknown, and then in the patient with a known diagnosis of SSc. Although critical ischemia is a relatively rare complication of RP, nonetheless a significant proportion of affected patients require surgery: the surgical approach to management is described in the second section.