Raynaud's phenomenon (RP) is an episodic digital ischemia provoked by
internal and/or external stressors. While the primary RP occurs indepe
ndently of other diseases and appears to be mediated by a vegetative d
ysbalance of the peripheral vasomotor tone, the secondary RP is often
part of an underlying disorder, especially of collagen-vascular diseas
es such as SLE or scleroderma. Vascular involvement in the pathogenesi
s of connective tissue diseases is well known but poorly understood. E
ndothelium injury by autoantibodies and circulating immune complexes m
ay play a central but not sufficient role in the pathogenesis of secon
dary RP. In this review, epidemiology and diagnosis of RP secondary to
connective tissues diseases are described and new pathogenic concepts
are discussed in view of current knowledge on endothelial function.