Raynaud's phenomenon and the vascular disease in scleroderma.
Raynaud's phenomenon (RP) is the most common feature of vascular disease in scleroderma. The diagnostic methods and mechanisms involved in the development of RP continue to be defined, and deficiency in the endothelial-dependent vasodilatory mechanism is suggested. Neuronal involvement in the pathogenesis of vasospasm is illustrated by a defective neuromediated vasodilatory mechanism in both spontaneous RP and vibration white-finger syndrome. The exact neuronal-related mechanism(s) is not known, but the development of scleroderma-like dermal changes following spinal cord injury suggests an important role for neuronal participation in the development of scleroderma. Mechanisms involved in endothelial injury are still not known, but the complement system may be involved, as suggested by the finding of decreased endothelial expression of the complement protective molecular system in scleroderma. Occurrence of vasculitis in the digital arteries in patients with digital ischemic lesions suggests that immune-mediated vascular injury occurs in the disease. The role of antiendothelial antibodies is questioned in view of the lack of endothelial specificity for the antibodies, but the association of anticentromere antibodies with vascular occlusion was confirmed. Therapeutic interventions stressing an individualized approach are recommended. Spinal cord stimulation and continued iloprost infusion are some of the newer proposed therapies for patients with difficult vascular complications.
Kahaleh
M B
MB
Division of Rheumatology, Medical College of Ohio, OH 43699, USA.
D006801:Humans; D011928:Raynaud Disease / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000473:pathology / Q000503:physiopathology