Secondary Raynaud’s Phenomenon

Abstract

Raynaud's phenomenon (RP) is an episodic digital ischemia provoked by internal and/or external stressors. While the primary RP occurs independently of other diseases and appears to be mediated by a vegetative imbalance of the peripheral vasomotor tone, the secondary RP is often part of an underlying disorder, especially of collagen-vascular diseases such as SLE or scleroderma. Vascular involvement in the pathogenesis of connective tissue diseases is well known but poorly understood. Endothelium injury by autoantibodies and circulating immune complexes may play a central but not sufficient role in the pathogenesis of secondary 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.