M Bashar Kahaleh1. 1. Division of Rheumatology, Department of Medicine, Medical College of Ohio, 1321 Glendale Avenue, Toledo, OH 43617, USA. bkahaleh@mco.edu
Abstract
PURPOSE OF REVIEW: Raynaud phenomenon is the earliest and most common clinical manifestations of scleroderma (systemic sclerosis). Therefore, Raynaud phenomenon offers the best window into the investigation of the early steps in the pathogenesis of systemic sclerosis. This review focuses on the differential diagnosis of Raynaud phenomenon, the transition of Raynaud phenomenon to systemic sclerosis, mechanisms and consequences of vascular injury and dysfunction in systemic sclerosis, and therapeutic options. RECENT FINDINGS: Careful clinical evaluation using a simple definition of Raynaud phenomenon is the most reliable and reproducible method in the diagnosis. Although the assessment of vascular function by noninvasive methods is still not sensitive enough for the evaluation and follow-up of individual patients, it helps in the differential diagnosis and in population studies. Progressive deficiency in vasodilatory capacity of the vessels is proposed as a mechanism of Raynaud phenomenon, particularly in systemic sclerosis. In addition, decreased fibrinolysis and enhanced coagulation pathways undoubtedly contribute to vascular dysfunction. The mechanism of endothelial injury is still elusive, yet endothelial apoptosis mediated by antiendothelial antibodies is the most attractive hypothesis now. Therapies directed at the vascular disease continue to focus on the alleviation of vascular spasm. However, immunosuppressive therapy may influence the levels of vascular injury markers and thus may have an effect on the vascular disease itself. SUMMARY: Continued progress in the investigation of the vascular aspects of scleroderma is described in this review. Immune involvement in the early stages of the disease and mechanism of vascular repair in advance cases are some of the highlights of last year's progress.
PURPOSE OF REVIEW: Raynaud phenomenon is the earliest and most common clinical manifestations of scleroderma (systemic sclerosis). Therefore, Raynaud phenomenon offers the best window into the investigation of the early steps in the pathogenesis of systemic sclerosis. This review focuses on the differential diagnosis of Raynaud phenomenon, the transition of Raynaud phenomenon to systemic sclerosis, mechanisms and consequences of vascular injury and dysfunction in systemic sclerosis, and therapeutic options. RECENT FINDINGS: Careful clinical evaluation using a simple definition of Raynaud phenomenon is the most reliable and reproducible method in the diagnosis. Although the assessment of vascular function by noninvasive methods is still not sensitive enough for the evaluation and follow-up of individual patients, it helps in the differential diagnosis and in population studies. Progressive deficiency in vasodilatory capacity of the vessels is proposed as a mechanism of Raynaud phenomenon, particularly in systemic sclerosis. In addition, decreased fibrinolysis and enhanced coagulation pathways undoubtedly contribute to vascular dysfunction. The mechanism of endothelial injury is still elusive, yet endothelial apoptosis mediated by antiendothelial antibodies is the most attractive hypothesis now. Therapies directed at the vascular disease continue to focus on the alleviation of vascular spasm. However, immunosuppressive therapy may influence the levels of vascular injury markers and thus may have an effect on the vascular disease itself. SUMMARY: Continued progress in the investigation of the vascular aspects of scleroderma is described in this review. Immune involvement in the early stages of the disease and mechanism of vascular repair in advance cases are some of the highlights of last year's progress.