‘Narrow-sense’ and ‘broad-sense’ vascular abnormalities of systemic sclerosis

Abstract

Systemic sclerosis (SSc) induces skin thickening and numerous symptoms involving the entire body. Collagen deposition, immune disorder, and vascular abnormalities is currently estimated to be three major causal factors involved in the respective conditions. Vascular abnormalities usually develop in the initial phase of this disease, and may exist in all phases; therefore, they markedly influence the patient's quality of life. This article reviews recent findings about 'narrow-sense' vascular lesions (including Raynaud's phenomenon, skin ulcers, nailfold bleedings, pitting scars, telangiectasia, and pulmonary hypertension) and 'broadsense' vascular lesions (such as calcinosis or erectile dysfunction). Affected blood vessels can be classified into arteriole/small artery and capillary blood vessels. Furthermore, pathological changes include the proliferation of the vascular endothelial or smooth muscle cells, lumen stenosis by collagen accumulation of the vascular intima, vasodilation or fragility, and apoptosis. There may be interaction between vascular lesions, autoimmune disorder, and collagen deposition. Thus, various symptoms of this disease may be controlled through the treatment of vascular lesions.

Calcinosis in Rheumatic Disease Is Still an Unmet Need: A Retrospective Single-Center Study

“…SSc patients had a higher frequency of DU history, which aligns with the proposal of a link between calcinosis and vasculopathy by Avouac et al, as their patient population with calcinosis was more likely to have a history of DU [ 4 ]. Masatoshi et al classified calcinosis as a “broad-sense” vascular lesion, proposing topical blood flow disturbance and vascular hypoxia as etiological factors [ 43 ]. This hypothesis finds support in a pilot study demonstrating reduced perfusion in the superficial skin layers of calcinotic areas compared to non-calcinotic areas [ 44 ].…”

The Involvement of Smooth Muscle, Striated Muscle, and the Myocardium in Scleroderma: A Review

“…SSc is defined by a widespread vasculopathy existing before the onset of tissue fibrosis [ 5 , 9 , 17 ]. It is one of the main pathogenic features involved in the development of Raynaud’s phenomenon, digital ulcers, pulmonary arterial hypertension, and scleroderma renal crisis [ 6 , 18 , 19 ].…”

Insights into the regulatory role of Plexin D1 signalling in cardiovascular development and diseases

“…92 Collagen deposition, immune disorders and vascular abnormalities are currently proposed to be the three major causal factors of SSc. 93 Vascular abnormalities usually develop during the initial phase of this disease and may exist in all phases. 94 The earliest stage of SSc is characterized by morphologic alterations in vessel walls, such as capillary enlargement, intimal proliferation and fibrosis.…”