Acute retinal artery occlusion in systemic sclerosis: A rare manifestation of systemic sclerosis fibroproliferative vasculopathy

Objectives: To describe three patients with systemic sclerosis (SSc) who developed acute unilateral blindness in the absence of any common etiologic factor for blindness. In one patient, the affected eye required enucleation and was examined histopathologically. Methods: Following identification of the first patient with retinal artery occlusion at the Scleroderma Center of Thomas Jefferson University, every patient evaluated at the Center from May 2001 to December 2010 was prospectively assessed for the development of acute unilateral blindness. Two additional cases were identified. Here, we describe the clinical features, laboratory and ancillary examinations of the three patients with SSc who developed acute unilateral blindness and present the histopathological examination of one eye enucleated from one of the patients. Results: Clinical and angiographic studies were consistent with acute retinal artery occlusion. The histopathological studies showed severe retinal ischemic atrophy and concentric narrowing and fibrosis of small retinal vessels. Conclusions: These findings suggest that acute retinal artery occlusion in these patients is a manifestation of the fibroproliferative vasculopathy characteristic of SSc.