Endogenous Intraocular Aspergillus Infection Associated With Systemic Vasculitis

We report the systemic, ocular, and investigational findings of a 51-year-old male patient with large-vessel vasculitis and presumed ocular Aspergillus infection. He presented with persistent fever with left-sided weakness of the upper and lower limb for the past 15 days accompanied by profound visual loss in the left eye. A neurological examination revealed a left-sided ataxic hemiparesis with a significant reduction of power in both upper and lower limbs with dysarthria. He underwent neuroimaging which revealed a fresh non-hemorrhagic infarct in the left thalamocapsular and left parieto-occipital regions, suggestive of a stroke. A positron emission tomography/computed tomography scan revealed a diffuse low-grade uptake (standardized uptake value = 3.6) associated with a circumferential wall thickening involving the ascending aorta, arch of the aorta, and descending and abdominal aorta, suggestive of active large-vessel vasculitis. On examination, his visual acuity was 6/9 unaided in the right eye and perception of light with inaccurate projection in the left. A dilated fundus examination revealed multiple hemorrhages, cotton-wool spots, and areas of retinal thickening associated with a hard exudate in the right eye. A similar picture was seen in the left eye with the additional findings of a large (1 DD x 1 DD) subretinal whitish-yellowish mass with surrounding superficial retinal hemorrhages in the superior quadrant. A B-scan through the subretinal revealed non-visualization of the retinal pigment epithelium-Bruchs membrane layer with a large subretinal mass with a basal hyporeflective area and hyperreflective areas superiorly, suggesting a choroidal Aspergillus infection with infiltration of the overlying retina but without vitreous seeding. He was treated with anti-epileptics, oral and injectable blood thinners, oral antihypertensives, and oral antidiabetic medication. Intravenous methylprednisolone 1 g once daily was administered for five days, followed by oral prednisolone in tapering doses. In view of the ocular findings and the presumed diagnosis of ocular aspergillus, oral voriconazole 400 mg daily was added. At the last follow-up, the subretinal mass had completely resolved with a residual area of pigmentary degeneration with loss of retinal layer differentiation on the B-scan. There was also a marked reduction in the hemorrhages and cotton-wool spots in either eye, suggesting a marked improvement of the retinal vasculitis. A larger dataset would be needed to confirm a potential causative role for systemic fungal infections in large-vessel vasculitis.