The evidence that generalized scleroderma (systemic sclerosis, SSc) is a vascular disease is compelling. Raynaud's phenomenon and subcutaneous edema are early manifestations, whereas vascular insufficiency from both arterial intimal scarring and microvessel obliteration in many organs compromises critical organ function, determines mortality and morbidity in the individual patient, and in association with fibrosis provides in the skin the fundamental pathogenetic rationale for dividing SSc into subsets that can empirically be shown to be prognostically meaningful.

Consider the extent of the clinical problem. Between 5% and 30% of adults worldwide can be shown to have the cold- and stress-induced exaggerated yet intermittent vasospasm called Raynaud's phenomenon (RP). Now well-established microscopic (nailfold capillaroscopy) and serologic (indirect immunofluorescence with rapidly dividing human cells such as HEp-2) laboratory determinations can select a small proportion of RP subjects (estimated to be 10% of all RP-positive and up to 50% of clinically referred RP-positive subjects) who, when capillary- and serology-positive, join the mild end of the spectrum of SSc with limited cutaneous systemic sclerosis. Thus, in the definable portion of adults who are Raynaud's-positive, and either capillary-positive or serology-positive, we have a group of subjects in which to study the natural history of at least limited cutaneous SSc and to interrupt the pathogenetic process when we understand how to intervene safely.<sup>7 Coffman J.D. Raynaud's phenomenon J Vasc Med Biol 1994 ;  5 : 259-269

Cliquez ici pour aller à la section Références, 24 LeRoy E.C., Medsger T.A. Raynaud's phenomenon: A proposal for classification Clin Exp Rheumatol 1992 ;  10 : 485-488

Cliquez ici pour aller à la section Références, 28 Maricq H.R., Weinrich M.C. Diagnosis of Raynaud's phenomenon assisted by color charts J Rheumatol 1988 ;  15 : 454-459

Cliquez ici pour aller à la section Références, 44 Summers L., LeRoy E.C. Raynaud's phenomenon in clinical dermatology Clinical Dermatology. Philadelphia: Lippincott-Raven (1995).  1-8

Cliquez ici pour aller à la section Références</sup>

It follows, therefore, that a detailed understanding of the vascular pathogenesis and its relationship to the fibrosis characteristic of SSc could directly influence our management and therapy of this difficult disease. Such a premise is the basis of this article.