Raynaud phenomenon (RP) is characterized by episodic vasospasm and occlusion of the digital arteries resulting in a well demarcated ischemic blanching of the involved digits. This may be followed sequentially by cyanosis and rubor. Tingling may occur during the reactive hyperemia phase (rubor).
|Connective Tissue Diseases
Systemic Sclerosis (SSc)
Mixed connective tissue disease (MCTD)
Polymyositis (PM) and Dermatomyositis (DM)
Systemic Lupus erythematosus (SLE)
Sjogren’s syndrome (SS)
Rheumatoid Arthritis (RA)
Polyarteritis nodosa (PAN)
Giant cell arteritis (GCA)
Microscopic polyangiitis (mPA)
Hand arm vibration syndrome (HAVS)
Occlusive/Structural Arterial diseases
Hypothenar hammer syndrome
Thromboangiitis Obliterans (Buerger’s Disease)
Thoracic outlet syndrome (TOS)
Carpal Tunnel Syndrome (CTS)
Cold agglutinin disease
Drugs and chemicals
Chemotherapy agents (bleomycin, vinblastin, cisplatin)
Complex regional pain syndrome (CRPS) type 1
Population-based studies estimate a prevalence of primary RP of 3-16 % among women and 1-6 % among men, the highest noted in the coldest geographic regions assayed. PRP is much more common than secondary RP, and usually presents in teens. The prevalence of SRP in SSc or scleroderma-overlap disorders is >90%, whereas in other CTD is about 20-30%. SRP usually occurs at older ages, often over the age of 40 and a male preponderance can be seen according to the etiology.
RP must be differentiated from processes characterized by persistent vasospastic ischemia. In the following disorders vasoconstriction is probably limited to arterioles and not the digital arteries.