Large vessel occlusive disease associated with CREST syndrome and scleroderma.

Significant attenuation of macrovascular involvement by bosentan in a patient with diffuse cutaneous systemic sclerosis with multiple digital ulcers and gangrene

“…The damaged small arteries characteristically show concentric intimal fibrosis, and less frequently medial thickening, fibrosis, and abnormal telangiectasia of the vasa vasorum in the tunica adventitia [7][8][9]. On the other hand, a growing body of evidence has demonstrated that macrovascular involvement, which is histopathologically characterized by medial thickening with circumferential luminal narrowing and occlusion with an acellular material [4], can be seen frequently in SSc patients with multiple digital ulcers and gangrene [2,3].…”

“…Although most of the attention in vascular involvement has been focused on microvessel injuries, which usually precede and trigger the fibrotic response in the skin and various internal organs [1], recent studies have demonstrated that macrovascular involvement was more frequently seen than previously believed, and that it contributed to the development of digital ulcers and gangrene in patients with SSc [2][3][4]. Digital ulcers and gangrene are two of the most frequently observed clinical manifestations, especially in diffuse cutaneous SSc (dcSSc), and strongly affect the functionality of hands and feet.…”

Estudo da macrovasculatura por ultra-sonografia Doppler na esclerose sistêmica

“…O fenômeno de Raynaud (FR), manifestação vascular mais freqüente, e as ulcerações digitais são exemplos comuns das alterações estrutural e funcional da vasculopatia na ES (8)(9)(10) . Estudos recentes também vêem evidenciando crescente acometimento macrovascular em pacientes esclerodérmicos (8,9,(11)(12)(13)(14)(15)(16)(17) , freqüentemente associados à significativa morbimortalidade durante a evolução da doença (18) . Apesar de a doença microvascular ser um dos marcadores da ES, o acometimento da macrovasculatura -doença macrovascular (DMV) -também está presente e não tem sido largamente reconhecido, embora alguns autores tenham sugerido esta associação (19,20) .…”

Vascular Disease in Scleroderma

“…Certainly, there is frequent and severe involvement of digital arteries [17]. Interestingly, evidence suggests a predilection for significant occlusive disease in the ulnar artery in patients with limited scleroderma that is associated with critical ischemic events [18][19][20]. One study has reported that measured increased arterial stiffness in scleroderma predicted more severe organ disease [21].…”