Correlation between Microvascular Damage and Internal Organ Involvement in Scleroderma: Focus on Lung Damage and Endothelial Dysfunction

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.

Multiparametric Skin Assessment in a Monocentric Cohort of Systemic Sclerosis Patients: Is There a Role for Ultra-High Frequency Ultrasound?

“…Systemic sclerosis (SSc) is a rare connective tissue disorder characterized by diffuse microangiopathy and immune dysregulation, pathogenic elements that ultimately lead to the most known feature of this disease, namely tissue fibrosis of skin and internal organs [ 1 , 2 ]. Skin thickening is one of the most evident and studied aspects of SSc since it can be easily analyzed and mostly because it has been widely demonstrated that a more extensive skin involvement correlates with more severe internal organ damage, poor prognosis and increased disability [ 3 , 4 ].…”

Cardiac involved and autopsy in two patients with systemic sclerosis: Two cases report

“…decreased blood flow) has been proved by many studies [ [6] , [7] , [8] , [9] , [10] ]. Nailfold papilloscopy (NVC) is a non-invasive, reliable, inexpensive, reusable and running-in method for determining structural microvascular injury in SSc [ 11 ]. Laser speckle contrast analysis (LASCA) is a new safety technology to quantify blood perfusion (BP) in different body regions of patients with SSc and healthy control group.…”

Biomarkers in Systemic Sclerosis: An Overview

“…ANA might not only represent biomarkers of disease but also play a pathogenic role through immune-mediated mechanisms and molecular mimicry. ANA (particularly anti-topoisomerase-I and anti-RNA polymerase III antibodies) appear to be transported within the cell by direct interaction with intercellular components and receptors, targeting intracellular topoisomerase and RNA polymerase by the corresponding antibodies [22,23,[49][50][51][52][53][54][55].…”

“…Supporting this evidence is the response of some SSc patients to B-cell-targeted therapies and the role of activated B cells in the success of allogeneic bone marrow transplantation for the treatment of SSc [24,25,[55][56][57][58][59][60]. In addition, SSc patients have macro-and microvascular damage and impaired peripheral blood perfusion [6,[48][49][50][51][52][53]. Nail fold video capillaroscopy (NVC) plays an important role because it is a noninvasive and reliable method to detect microvascular involvement.…”