A 55-year-old woman was admitted to our department with complaints of progressive vision loss in the left eye over the course of 3 days. The patient reported weight loss, asthenia, and polyarthralgia with progressive weakness persisting for 2 months. Four weeks earlier, she was referred to the local hospital because she experienced persistent fever and increasing shortness of breath that had not responded to amoxicillin–clavulanate treatment; she was found to have pneumonia and was empirically treated with various antibiotics. She also developed an episode of sudden onset, painless reduced vision in the right eye after admission. She had presented to the emergency department 2 months ago with complaints of fever and cough with mucopurulent expectoration, and she had been treated with amoxicillin–clavulanate.
The patient was referred to our Ophthalmology Department by the rheumatologist who provided her with further management. Entering examination revealed no perception of light in her left eye while the visual acuity (VA) of the right eye was 20/60. Anterior segment appeared unremarkable. Intraocular pressure was normal. Dilated fundoscopy of the left eye demonstrated a cherry-red spot at the macula with edematous retina, characteristic of CRAO (Fig. 1a). The right fundus showed a slightly pale retina in the posterior pole (Fig. 1b). The patient was not able to have fluorescein angiography and indocyanine green angiography due to her bad general conditions. On physical examination, she was febrile and short of breath; her blood pressure was also high. Her cardiopulmonary examination revealed increased sounds at the right lung base. In her musculoskeletal examination, there was swelling and sensitivity to palpation in the proximal interphalangeal joints of the right hand and also upon her shoulders, left ankle, and knee. Her past medical history included hypertension and chronic venous insufficiency. The initial patient workup included complete blood count (showing anemia, neutrophilic leukocytosis, and thrombocytosis); biochemistry results showed an erythrocyte sedimentation rate of 5 mm in the first hour and a CRP higher than 90 mg/l, a blood urea nitrogen level of 28 mg/dL, and a creatinine level of 2.5 mg/dL, but normal electrolytes and liver function. Urinalysis revealed moderate hematuria and mild pyuria. Chest X-ray was negative for pneumonia (Fig. 2). Possibility of systemic vasculitis was kept in mind, and intravenous methyprednisolone pulses were started (three pulses of 0.5 g each). Fluorescein angiography taken a few days later disclosed delayed retinal arterial filling in both eyes and nonperfusion through affected cilioretinal artery in the left eye and, surprisingly, affected choroidal blood flow with choroidal filling defect in the posterior pole of the right eye (Fig. 3a) and in peripheral areas of the left eye (Fig. 3b), rarely seen in patients with CRAO [9].
Additional blood tests revealed anti-nuclear antibody titer of 1:40, anti-SSA/Ro-antibody-positive, negative hepatitis and HIV antibodies, and also progressively increasing serum creatinine levels with a creatinine clearance of 18 mL/min. Therefore, the patient developed renal failure. The diagnosis of WG was supported by strongly positive C-ANCA anti-PR3 antibodies confirmed by kidney biopsy that showed focal and segmental necrotizing vasculitis of the medium-sized arteries (Fig. 4). Computed tomography (CT) of the brain and orbits was normal. CT scan of the chest, abdomen, and pelvis revealed pleural effusion, perirenal hematoma, and splenic infarction (Fig. 5a, b). The patient was aggressively treated with cyclophosphamide for six consecutive cycles, which resulted in a total cumulative dose of 6.8 g. Concomitant administration of corticosteroid (1 mg/kg/day oral prednisone) was used, and the patient gradually improved with radiographic clearing of pulmonary abnormalities and normalization of laboratory studies. To maintain remission, oral azathioprine (2 mg/kg/day) and prednisone (15 mg/day) were administered and were then gradually tapered. Two years after diagnosis, VA remains 20/60 in the right eye with no light perception in the left eye. The funduscopy shows bilateral optic disc pallor (Fig. 6a, b). Since then, the patient has been clinically stable, as seen in the OCT (Fig. 7a, b).