Retinal vessels thrombosis as onset manifestation of systemic sclerosis: 3 clinical cases.

Abstract

To the Editor: Posterior ocular segment involvement in systemic sclerosis (SSc) is rare. To the best of our knowledge, the retinal vascular occlusion presenting at onset of SSc has never been reported in the literature. We describe 3 cases of patients with retinal thrombosis without apparent risks of ocular disease, followed by fully SSc typical manifestations. Of note, all 3 patients fulfilled the new 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for the diagnosis of SSc at onset, while the previous 1980 ACR criteria were satisfied long after. In SSc, the most frequent ocular manifestations involve anterior segment, such as eyelid skin fibrosis and keratoconjunctivitis sicca. Posterior segment involvement is less common, and findings similar to those observed in arterial hypertensive retinopathy, such as cotton wool spot, optical disc edema, and hemorrhages, have been reported1,2. Nevertheless, abnormal ocular findings are frequently detected by fluorescein angiography (FA), and posterior segment microvessel damage and pigment epithelium layer atrophy are shown in up to 50% of patients with … Address correspondence to Dr. F. Iannone, Rheumatology Unit, Policlinico, Piazza G. Cesare 11, 70124 Bari, Italy. E-mail: florenzo.iannone{at}uniba.it