Background/Purpose: To analyze the morphological and functional abnormalities of the microcirculation through widefield nailfold capillaroscopy (NFC), videocapillaroscopy and cutaneous microvascular blood flow measurement before and after cold stimulus, associated with vascular injury markers in patients with early systemic sclerosis (SSc), compared to primary Raynaud’s phenomenon (PRP), definite SSc, and healthy controls.

Methods: Eighty patients with definite SSc (American College of Rheumatology criteria), 46 patients with early SSc (2001 LeRoy’s criteria), 40 PRP patients, and 45 controls were included. The morphological abnormalities were evaluated through widefield nailfold capillaroscopy (NFC) (10-25x magnification), and videocapillaroscopy (200x magnification). The following parameters were analyzed in eight digits of the hands (excluding the thumb) by widefield NFC and videocapillaroscopy: number of capillaries/mm, enlarged, giant and ramified capillaries, microhaemorrhages, and avascular score. A score based on a semiquantitative rating scale (score 0–3) was used for each videocapillaroscopy parameter. Patients with scleroderma pattern were distributed into three patterns: early, active and late. Fingertip blood flow (FBF) was measured using laser Doppler imaging (LDI) at baseline and during 30 minutes after cold stimulus. Serum endothelin-1 (ET-1), von Willebrand factor (vWF) and transforming growth factor beta-1 (TGF-β1) were measured by ELISA.

Results: Patients with early SSc showed significantly higher number of capillaries/mm, and lower enlarged, giant capillaries and avascular score compared to patients with definite SSc in widefield NFC and videocapillaroscopy (p<0.001). Besides the number/score of enlarged capillaries, microhaemorrhages and avascular score were higher in patients with early SSc compared to patients with PRP and controls in both methods (p<0.001). Ninety-six percent of patients with definite SSc, and 76% of early SSc patients presented the scleroderma pattern using videocapillaroscopy. The early pattern was most frequently found among early SSc patients (37% in early SSc vs 17% in definite SSc, p<0.01). The active pattern was found in 63% of early SSc and in 60% of definite SSc. Interestingly, the late pattern was not observed in patients with early SSc, but was observed in 23% of definite SSc patients. FBF values before and after cold stimulus were significantly higher in controls compared to PRP, early SSc and definite SSc (p<0.001), with no difference between early and definite SSc. Serum levels of ET-1, vWF activity and TGF-β1 were similar between early and definite SSc.

Conclusion: Early SSc patients showed functional changes and serum vascular markers levels similar to patients with established disease. Nonetheless, the morphological changes were less severe in early SSc, suggesting a progression of the peripheral microangiopathy along different phases of the disease. These findings may have important implications in the management of patients with early disease.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/microvascular-abnormalities-in-patients-with-early-systemic-sclerosis-less-severe-morphological-changes-compared-to-patients-with-definite-disease/