From VEDOSS to established systemic sclerosis diagnosis according to ACR/EULAR 2013 classification criteria: a French-Italian capillaroscopic survey.

Objectives: Nailfold capillaroscopy (NC) shows microcirculatory abnormalities in systemic sclerosis (SSc). The inclusion of NC specific abnormalities increases the sensitivity of both 2013 ACR/EULAR and VEDOSS (Very Early Diagnosis of Systemic Sclerosis) classification criteria. We aimed to detect NC features able to predict progression toward established SSc in VEDOSS patients.

Methods: Sixty-six VEDOSS patients were enrolled. They had a clinical follow-up and underwent NC once a year, considering morphological parameters, appropriate pattern and semi- quantitative rating scale.

Results: In a mean follow-up time of 31 months, 21 patients progressed into SSc (P = Progressors), while 45 were "Non Progressor" (NP). Comparing NC basal features of both groups, significantly larger loop diameter and apex width, higher haemorrhage and NC scores were found in P respect to NP patients. When comparing NC features of P patients who progressed within one year (FP = Fast progressor), loop diameter and apex width were significantly higher compared to all VEDOSS subjects. Each unit increase of apex width was associated with an increasing risk of 1% for developing SSc and the cut-off value of 103 μm showed a positive predictive value of 56% and a negative predictive value of 71%.

Conclusions: We describe NC findings in VEDOSS patients, identifying those suggesting a progression into established disease. These findings must be regarded as possible predictive risk factor to develop SSc and can also be of relevance in the detection of those cases with a faster development. Thus NC seems to have a diagnostic and prognostic role in VEDOSS cases.