Background/Purpose: A primary endothelial cell dysfunction is thought to be involved in systemic sclerosis (SSc)-associated fibroproliferative vasculopathy of the microcirculation and small arterioles, even in sites not affected by fibrosis.  Since, (i) the role of resident fibroblasts in the pathologic modifications and vascular wall remodeling is still unclear, and (ii) a fibroblast–free environment, such as the retina, provides a unique opportunity to assess the microcirculation, we systematically evaluated the retinal vessels in patients with SSc.

Methods: Digital retinal images were obtained from both eyes of 93 consecutive patients with fully characterized SSc and 29 healthy controls effectively matched 1:1 for age and gender with 29 selected patients without diabetes, hypertension history, or anti-hypertensive treatment. Internal microvascular calibers (erythrocyte column width in micro-m) by central retinal arteriolar and venular equivalents and arteriolar to venular ratio were measured using validated software. 

Results: Arterioral and venular calibers were similar in patients and their matched controls (mean±SEM of 187±2 vs 184±3, p=0.444 and 211±2 vs 216±3, p=0.314, respectively).  Both arterioral and venular calibers and their ratio in SSc patients were not associated with disease duration, the extent of skin involvement, the presence of pulmonary fibrosis, digital ulcers or absorptions, amputations, digital capillaroscopic findings, inflammatory indices, or autoantibodies.   

Conclusion: The strong evidence that retinal microcirculation is spared in SSc suggests that the fibroproliferative vasculopathy depends on resident fibroblasts and/or other specific cellular or soluble factors not present in the retinal environment.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/could-a-fibroblast-free-environment-protect-the-microcirculation-in-systemic-sclerosis-evidence-from-retinal-vascular-imaging-research/