Structural capillary changes in systemic sclerosis (SSc) may be easily investigated and quantified by nailfold videocapillaroscopy (NVC), and in recent years there have been identified disease-specific progressive capillary abnormalities and related specific capillaroscopic patterns of microangiopathy (“early”, “active, “late” scleroderma NVC patterns) (1). Severity of organ involvement in SSc may be evaluated robustly by the disease severity scale of Medsger which provides a scale with progressive categories 0–4 for nine organ systems (general, peripheral vascular, skin, joint, muscle, gastrointestinal tract, lung, heart and kidney) (2). For each of the nine organ systems association between baseline capillaroscopy patterns and future severe organ involvement has been recently shown. In particular, a recent study has shown 13% of patients with normal baseline NVC pattern, while 20%/33%/63% of patients with “early“/“active“/“late” scleroderma pattern developed future severe organ disease (3). Another longitudinal study demonstrated a dynamic transition of microvascular damage through different NVC patterns of microangiopathy in almost 50% of SSc patients: clinical symptoms progressed in accordance with the NVC morphologic changes in 60% of the SSc patients (4). Therefore, it is recommended that patients exhibiting rapid progression from the “early” to the “active” NVC pattern (<1 year) should be monitored closely, since the evidence suggests that they are at risk of rapid progression to the advanced (“late“) NVC pattern of microangiopathy that is associated with further clinical manifestations of SSc. Finally, in a very recent study, regarding the prognostic value of NVC progressive capillary abnormalities, the long term treatment with ET-1 receptor antagonist in combination with iloprost in SSc patients affected by digital ulcers (DU), was found to interfere with progression of nailfold microvascular damage by increasing the capillary number, as assessed by NVC over a 3-year followup period (5). The capillary number increase was found statistically significant when compared to SSc patients in monotherapy with iloprost, as wells as was detected a progressive significant increase of angiogenesis (p < 0.01). The predictive role of NCV, at least for SSc DU, is also the result of a large multinational European study (CAP study), first reported during the EULAR 2013 Conference in Madrid. Finally, NCV prognostic changes observed during treatment of SSc (i.e. with cyclosporin, cyclophosphamide, rituximab) have been reported in the lest years and early predictive indexes have been validated (6,7).
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Corresponding Maurizio Cutolo. 1Research Laboratory and Academic Unit of Clinical Rheumatology, Dept Internal Medicine, Genoa University-I e-mail: email@example.com
Disclosure of Interest None Declared
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