Different patterns of endothelial cell activation in renal and pulmonary vascular disease in scleroderma

摘要

Abnormal endothelial cell function is implicated in thedevelopment of scleroderma, an in major life- threateningcomplications of the disease. The nature of the stimulus leading toabnormal endothelial cell function in scleroderma, scleroderma renalcrisis, and scleroderma-associated pulmonary hypertension wasinvestigated by measurement of soluble adhe- sion molecule, shed byactivated endothelial cells, in sera from patients with theseconditions. In sclero- derma renal crisis, mean levels of solubleE-selectin (p<0.05 limited scleroderma, p<0.0001 diffusescleroderma), sVCAM-1 (soluble vascular cell adhe- sion molecule-1)(p<0.05 limited scleroderma, P<0.05 diffuse scleroderma), and sICAM-1(soluble Intercellular adhesion molecule-1) (p,0.0001 lim- itedscleroderma, p<0.0001 diffuse scleroderma) were raised, supporting amodel of endothelial cell activation in this complication.