Abstract

Background: Longitudinal study to define a scoring system to quantify the specific capillary abnormalities, as observed by capillary microscopy in systemic sclerosis (SSc).

Methods: Ninety patients with SSc were evaluated by nailfold videocapillaroscopy for an average of 72 (SD 23) months. Enlarged and giant capillaries, haemorrhages, loss of capillaries, disorganisation of the microvascular array, and capillary ramifications were the evaluated parameters identifying the “scleroderma patterns”. A semiquantitative rating scale to score these altered microvascular parameters was adopted (score 0–3). A “microangiopathy evolution score” (sum of three scores: loss of capillaries, disorganisation of the microvascular array and capillary ramifications) was also selected to assess the progression of the vascular damage.

Results: At the end of the follow-up, the score for each nailfold videocapillaroscopy parameter significantly changed. The microangiopathy evolution score significantly increased in 53 of 90 patients (59%) indicating a worsening of the microangiopathy. On the contrary, 22 patients (24%) showed a significant decrease of the evolution score suggesting an improvement of the microangiopathy and no changes were detected in 15 patients with SSc (17%).

Conclusions: The capillaroscopic score was found to be a sensitive tool to quantify and monitor the SSc microvascular damage. Furthermore, the microangiopathy evolution score might be used to survey the evolution of the microvascular damage, as the relative scores increase during the progression of the SSc.