[Vascular pathology in systemic scleroderma].

The mechanisms of vascular lesions in systemic scleroderma (SSC) are still little studied with the current comprehensive investigations being focused on this issue. The results of a study dealing with the structural-and-morphological and molecular reasons of sclerodermic micro-angiopathy as compared with the variations and pattern of the clinical disease course are summarized in the article. The structural capillary changes were evaluated on the basis of the results of a wide-field video-capillaroscopy of the nail bed (CNB). The level of soluble adhesion molecules VCAN-1, ICAM-1 and P-selectine determined by the quantitative immune-enzyme assay described the vascular endothelium condition. Morphological examinations of dermal samplings included an identification of the lymphocytic composition of infiltrates by applying the mononuclear antibodies to markers T (CD3, CD4, CD8) and B (CD20) of lymphocytes and detection of the endothelial activation by applying the mononuclear antibodies to intercellular adhesion-1 molecule (ICAM-1). The conducted investigations revealed the structural capillary changes in all SSC patients; the nature of such changes is closely related with a clinical variation and course of the disease. The morphological signs of micro-angiopathy were detected in 98% of patients including at the early disease stage. A more pronounced perivascular infiltration with predominance of CD4+ T-lymphocytes was observed and expression of ICAM-1 to the endothelial cells was registered more often in an active disease course. Higher levels of VCAM-1, ICAM-1 and P-selective in blood were found in 80%, 45% and 48% of patients, respectively. Correlations of VCAM-1 with an activity and a progressing disease course were established. Therefore, the serological and morphological signs of vascular lesions reflect an intensity degree of sclerodermic micro-angiopathy and correlate with an SSC clinical course.