Central retinal artery occlusion in Wegener's granulomatosis: a diagnostic dilemma

Abstract

PurposeTo report a case of central retinal artery occlusion (CRAO) in a patient with biopsy-verified Wegener's granulomatosis (WG) with positive C-ANCA.MethodsA 55-year-old woman presented with a 3-day history of acute painless bilateral loss of vision; she also complained of fever and weight loss. Examination showed a CRAO in the left eye and angiographically documented choroidal ischemia in both eyes.ResultsThe possibility of systemic vasculitis was not kept in mind until further studies were carried out; methylprednisolone pulse therapy was then started. Renal biopsy disclosed focal and segmental necrotizing vasculitis of the medium-sized arteries, supporting the diagnosis of WG, and cyclophosphamide pulse therapy was administered with gradual improvement, but there was no visual recovery.ConclusionCRAO as presenting manifestation of WG, in the context of retinal vasculitis, is very uncommon, but we should be aware of WG in the etiology of CRAO. This report shows the difficulty of diagnosing Wegener's granulomatosis; it requires a high index of suspicion, and we should obtain an accurate medical history and repeat serological and histopathological examinations. It emphasizes that inflammation of arteries leads to irreversible retinal infarction, and visual loss may occur

Conjunctivitis as a manifestation of Wegener's Granulomatosis

“… 11 Tarsal conjunctivitis can be associated with nasolacrimal duct obstruction and subglottic stenosis. 5 , 11 Robinson et al reported conjunctivitis in 16% of Wegener patients. 11 …”

“…The most common manifestations are orbital disease (18/140, 15%) followed by scleral (7%), episcleral (3.5%), corneal (8%), and nasolacrimal (7%) abnormalities. 5 , 8 Conjunctivitis in GPA may be ulcerative and necrotic and can cause cicatricial changes in the ocular surface. 9 , 10 Areas of necrosis, active fibrovascular changes, and fibrovascular scars can be seen with tarsal conjunctivitis.…”

“…Keratitis in GPA is associated with the presence of an autoantibody specific against cytokeratin-3. 5 It manifests as interstitial and PUK. 12 In the condition of PUK with scleritis, one important differential diagnosis is GPA.…”

“… 4 Ophthalmic manifestations are common in the disease (up to 58% of patients). 5 Cases of eyelid and conjunctival involvement as the initial manifestations of the disease are rare. 6 …”

Simultaneous unilateral presentation of three different ocular manifestations of granulomatosis with polyangiitis

“…Ophthalmologists may be the first clinicians to encounter GPA patients because ocular manifestations, particularly necrotizing scleritis, may be the first sign of the disease. [ 5 ]…”