A Case of Progressive Optic Neuropathy in a Patient with Wegener’s Granulomatosis

Nova Southeastern University, Davie, Florida, USA

Introduction: We describe a case of a patient with Wegener’s Granulomatosis (WG) who developed progressive optic atrophy and worsening of vision despite treatment. Case presentation: A 46 year old Hispanic male presented with complaints of blurry vision OD>OS. He had been diagnosed with WG 3 months earlier and after experiencing multiple symptoms, was stabilized on prednisone. VA was 20/15 in each eye, C/D ratios were .20 OU with sectoral pallor OD, moderate retinal nerve fiber layer (RNFL) loss OD on OCT, and retinal vascular changes OU. Visual fields showed generalized depression OD and arcuate defect OS. At a 5-month follow up visit, he complained of worsening vision, and VA had worsened to 20/40 OD. Discs in both eyes showed increased cupping, increased pallor, and markedly thinner RNFL. Deviation from the Expected: We know of no reported cases of progressive optic atrophy over the course of several months in a systemically stable WG patient. Discussion: WG is a rare, multisystem autoimmune disease that causes inflammation of blood vessels, leading to tissue necrosis. Most commonly affecting the respiratory tract and kidneys, without treatment prognosis is poor. Ocular involvement occurs in 50-60% of cases, potentially affecting virtually every part of the eye. Optic atrophy, secondary to optic neuritis may be caused by vasculitis, inflammation from adjacent sinuses or compression by intraorbital granuloma. Conclusion. – WG may lead to progressive vision loss over a relatively short period of time despite the absence of systemic symptoms. Co-management with the other patient providers is essential.