Posterior uveitis: A rare systemic association

Author: Francisco Manuel Hermoso Fernandez (Spain)

Co-authors: María Cruz Rojo, Jose Luis García Serrano, Maria Carmen Gonzalez Gallardo

Purpose

To report a case showing a very rare association of bilateral retinal vasculitis (RV) and systemic sclerosis sine scleroderma (ssSSc).

Setting/Venue

Department of Ophthalmology of the San Cecilio University Hospital of Granada, Spain.

Methods

Observational case report. The description of a 40-year-old woman with a bilateral retinal vasculitis in association with systemic sclerosis sine scleroderma. We portray the findings of the visual acuity, fundus examination, OCT and angiography. We also used the PubMed database to find other prospective or retrospective studies.

Results

A 40-year-old woman presented at our Hospital for blurry vision with her left eye (OS). Her best-corrected visual acuity was 20/20 in both eyes. Funduscopy examination showed bilateral occlusive vasculitis with proliferative retinopathy and vitreous hemorrhages. Retinography showed temporary sheathed and some occluded veins, surrounded by large number of retinal hemorrhages in both eyes. In fluorescein angiography were found areas of not perfused retina, also with telangiectasia and staining of retinal vessel’s walls. We found isolated microaneurysms in these ischemic areas. The ophthalmological treatment was confluent photocoagulation of the ischemic retina of both eyes, and vitrectomy of the right eye to treat his vitreous hemorrhage. The systemic treatment was corticosteroids and Adalimumab during two years; afterwards the stability was achieved. There was a lack of response to cyclosporine and azathioprine. The diagnosis comes after presenting: anticentromere antibodies, scleroderma pattern on capillaroscopy and esophageal manometry showing esophageal dysmotility with absent contractility.

Conlusions

'- RV can precede the development of ssSSc by several years. - Systemic sclerosis sine scleroderma can occur with retinal vasculitis, and that may be underdiagnosed. - Positivity for anticentromere antibodies can alert us to look for possible systematic and organic involvement of the disease.

Financial Disclosure

NO FINANCIAL DISCLOSURE