Microvascular abnormalities in patients with early systemic sclerosis: less severe morphological changes than in patients with definite disease

Abstract

Objectives: To evaluate the morphological and functional abnormalities of the microcirculation associated with markers of vascular injury in patients with early systemic sclerosis (SSc).

Method: Forty-six patients with early SSc were compared with 80 patients with definite SSc, 40 patients with primary Raynaud’s phenomenon (PRP), and 45 healthy subjects. Widefield nailfold capillaroscopy (NFC) (10–25 × magnification), videocapillaroscopy (200 × magnification), and laser Doppler imaging (LDI) assessment were performed in all participants. The number of capillaries/mm, enlarged, giant and ramified capillaries, microhaemorrhages, and the avascular score were determined by widefield NFC and videocapillaroscopy. Fingertip blood flow (FBF) was measured using LDI before and after cold stimulus (CS). Serum endothelin-1 (ET-1), von Willebrand factor (vWF), and transforming growth factor beta-1 (TGF-β1) were measured by enzyme-linked immunosorbent assay (ELISA).

Results: Upon both widefield NFC and videocapillaroscopy, patients with early SSc showed significantly higher numbers of capillaries/mm, lower enlarged and giant capillaries, and a lower avascular score than definite SSc patients (p < 0.001). They also had more enlarged capillaries, microhaemorrhages and a higher avascular score compared to PRP and controls (p < 0.001). FBF before and after CS were significantly higher in controls than in PRP, early SSc, and definite SSc patients (p < 0.001), with no difference between early and definite SSc. Serum levels of ET-1, vWF, and TGF-β1 were similar between early and definite SSc patients.

Conclusions: Early SSc patients showed functional changes and vascular injury marker levels similar to patients with established disease. Nonetheless, the morphological changes were less severe in early SSc, thus providing an opportunity for further prevention of vasculopathy progression.

Acknowledgements

This study was supported by the State of São Paulo Research Foundation (FAPESP grant 2010/51667-7, 2010/12613-9).