Advances in nailfold capillaroscopic analysis in systemic sclerosis

Abstract

Systemic sclerosis is an autoimmune connective tissue disease characterized by early and persistent microvascular impairment which leads to functional and organic manifestations, with progressive fibrosis of the skin and internal organs. Morphological and functional assessment of the peripheral microvasculature is a must, not only for diagnosis but also for the prognosis and therapeutical follow-up of systemic sclerosis patients, as reported in recent studies. Nailfold videocapillaroscopy is the validated technique for the study of scleroderma microangiopathy as it is able to detect peripheral microvascular morphology and both classify and score the capillary abnormalities into different microangiopathy patterns ('Early', 'Active' and 'Late'). Indeed, the possibility to early diagnose and follow the microvascular changes and the safety of the technique have made nailfold videocapillaroscopy a mandatory tool for patient evaluation and included its assessment in the new systemic sclerosis classification criteria. Important links between nailfold videocapillaroscopy patterns and systemic sclerosis clinical manifestations have been described.

Slit2/Robo4 axis may contribute to endothelial cell dysfunction and angiogenesis disturbance in systemic sclerosis

“…As displayed in figure 1C, circulating Slit2 levels were significantly higher in both patients with SSc and patients with VEDOSS than in healthy controls (p=0.002 and p=0.001, respectively). We also evaluated the possible correlation of Slit2 levels with the nailfold videocapillaroscopy (NVC) pattern27 and the presence/absence of digital ulcers in SSc, as measures of peripheral microvascular involvement severity. Either patients with SSc with early/active NVC pattern or those with late NVC pattern had increased Slit2 levels in respect to controls (p=0.009 and p=0.024, respectively) (figure 1D).…”

Vascular Leaking, a Pivotal and Early Pathogenetic Event in Systemic Sclerosis: Should the Door Be Closed?

“…Non-invasive investigational studies support a progressive hyperpermeability of microvasculature in SSc. Nailfold videocapillaroscopy (NVC) is one of the major components in the 2013 classification of SSc ( 74 ), demonstrating progressive loss of the peripheral vascular network, dilatation of capillaries, deficient vascular repair and inadequate angiogenesis ( 75 ). While NVC is efficient for measuring morphological microvascular changes (microangiopathy), other methods, such as Laser Doppler, thermography or Near Infrared Spectrometry ( 76 ), are essential for the assessment of functional blood flow abnormalities.…”

Sidestream Dark Field (SDF) imaging of oral microcirculation in the assessment of systemic sclerosis

“…As microvascular dysfunction is regarded an initial pathological event, nailfold capillaroscopy has been included in this classification criteria. In these images, cap-illary loops appear dilated (damaged from inflammatory re actions) and are lost as a result of progressive destruction (Ruaro et al, 2018). Sidestream Dark Field (SDF) imaging is also a non-invasive imag-ing technique that has potential to quantify physiological parameters such as capillary density in mucosal microcirculation.…”