Correlation between Microvascular Damage and Internal Organ Involvement in Scleroderma: Focus on Lung Damage and Endothelial Dysfunction
Mario D’Oria, Ilaria Gandin, Riccardo Pozzan, Michael Hughes, Sandro Lepidi, Francesco Salton, Paola Confalonieri, Marco Confalonieri, Stefano Tavano, Barbara Ruaro
Abstract
BACKGROUND: Systemic sclerosis (SSc) is an incurable connective tissue disease characterized by decreased peripheral blood perfusion due to microvascular damage and skin thickening/hardening. The microcirculation deficit is typically secondary to structural vessel damage, which can be assessed morphologically and functionally in a variety of ways, exploiting different technologies. OBJECTIVE: This paper focuses on reviewing new studies regarding the correlation between microvascular damage, endothelial dysfunction, and internal organ involvement, particularly pulmonary changes in SSc. METHODS: We critically reviewed the most recent literature on the correlation between blood perfusion and organ involvement. RESULTS: Many papers have demonstrated the link between structural microcirculatory damage and pulmonary involvement; however, studies that have investigated correlations between microvascular functional impairment and internal organ damage are scarce. Overall, the literature supports the correlation between organ involvement and functional microcirculatory impairment in SSc patients. CONCLUSIONS: Morphological and functional techniques appear to be emerging biomarkers in SSc, but obviously need further investigation.