Abstract
Nailfold microvascular impairment represents an early feature of systemic sclerosis (SSc) and its progression through different patterns of capillary damage and their validated scoring, is evaluable by nailfold videocapillaroscopy (NVC) in a safe and reliable manner.
The presence of specific morphological microvascular alterations at the NVC (i.e., presence of giant capillaries) is fundamental and mandatory for the early diagnosis of SSc, together with the presence of the Raynaud's phenomenon.
Furthermore, a recent longitudinal study showed a dynamic transition of microvascular damage through different NVC patterns of microangiopathy in almost 50% of SSc patients and clinical symptoms progressed in accordance with the NVC morphologic changes in 60% of the SSc patients.
A pilot study was the first demonstrating an association between baseline NVC patterns and future severe, peripheral vascular and lung involvement with stronger odds according to worsening scleroderma patterns. Prognostic indexes for digital trophic lesions, especially for daily use in SSc clinics and simply limited to the mean score of capillary loss are now validated.
Very recently, it has been described that efficacious potentially disease modifying therapies in SSc may interfere with progression of nailfold microvascular damage, as assessed by NVC, over long term at least in presence of digital ulcers. NVC is a safe and reliable tool for the early diagnosis of SSc and the different NVC scleroderma patterns have a predictive value for the clinical complications of the disease.
Keywords: Early diagnosis, endothelin-1 receptor antagonist, nailfold capillaroscopy, prediction, scleroderma pattern, systemic sclerosis.