Endothelial Dysfunction in Systemic Lupus Erythematosus and Systemic Sclerosis: A Common Trigger for Different Microvascular Diseases

Abstract

This review describes the complex interplay between inflammation, vasculopathy and fibrosis that involve the heart and peripheral small vessels, leading to endothelial stiffness, vascular damage, and early aging in patients with systemic lupus erythematosus and systemic sclerosis, which represents two different models of vascular dysfunction among systemic autoimmune diseases. In fact, despite the fact that diagnostic methods and therapies have been significantly improved in the last years, affected patients show an excess of cardiovascular mortality if compared with the general population. In addition, we provide a complete overview on the new techniques which are used for the evaluation of endothelial dysfunction in a preclinical phase, which could represent a new approach in the assessment of cardiovascular risk in these patients.

Keywords: endothelial dysfunction; microvascular disease; systemic lupus erythematosus; systemic sclerosis; techniques of assessment.

FIGURE 1

Systemic lupus erythematosus and systemic sclerosis as paradigmatic diseases in showing multiple factors involved in cardiovascular complications related to inflammatory and autoimmune processes. ACA, anti-centromere antibodies; anti-dsDNA, anti-double stranded-DNA antibodies; AECA, anti-endothelial cell antibodies; aPL, anti-phospholipid antibodies; anti-topo-I, anti-topoisomerase-I antibodies; C’, complement; CV, cardiovascular; DU, digital ulcers; ECs, endothelial cells; EPCs, endothelial progenitor cells; ET1, endothelin1; IFN, interferon; NETs, neutrophil extracellular traps; NO, nitric oxygen; ox-LDL, oxidized low-density lipoprotein; PAH, pulmonary arterial hypertension; proinflamm-HDL, proinflammatory high-density lipoprotein; ROS, reactive oxygen species; RP, Raynaud’s phenomenon; SLE, systemic lupus erythematosus; SRC, scleroderma renal crisis; SSc, systemic sclerosis; Tang, angiogenic T cells; VEGF, vascular endothelial growth factor. Created with BioRender (academic license).