Clinical Images: Acute vision loss due to granulomatosis with polyangiitis

Abstract

A 70-year-old man presented with progressive hearing loss and persistent postnasal drip for five months. Despite starting antibiotics, the patient's condition remained refractory. The patient suddenly experienced vision loss two weeks before presentation. Physical examination revealed complete blindness, positive relative afferent pupillary defect in the right eye, and hearing loss. Ophthalmoscopy revealed no abnormalities. Audiometry demonstrated severe bilateral mixed hearing loss. Laboratory findings showed elevated C-reactive protein level (5.94 mg/dL), erythrocyte sedimentation rate (115 mm/h), and positive proteinase 3 antineutrophil cytoplasmic antibody level (89.7 IU/L). Antinuclear antibody, T-SPOT.TB test, and blood culture results were negative. Angiotensin-converting enzyme, lysozyme, serum IgG4, interleukin-2 receptor, and all tumor marker levels were normal. Axial (A, arrows) and coronal (B, arrow) T2-weighted enhanced magnetic resonance imaging showed high intensity and contrast effect around the right optic nerve, the so-called “tram track sign” and “donut sign,” which indicated optic perineuritis. Furthermore, sinusitis was detected (C, arrows). Computed tomography showed poor air-containing and soft shadows in the bilateral mastoid antrums (D, arrows) and fluid accumulation in left maxillary sinus (D, arrowhead) that suggested otitis media and sinusitis. A biopsy specimen from the right maxillary sinus revealed lymphocyte and plasma cell infiltration around the capillaries in hematoxylin and eosin staining (E, arrowheads), and Elastica-van Gieson staining showed elastic fiber disruption in the vessel wall (F, arrowheads), which indicated small-vessel vasculitis. Thus, granulomatosis with polyangiitis (GPA) was diagnosed. Methylprednisolone at 1,000 mg was initiated, followed by intravenous administration of rituximab at 500 mg weekly and gradual tapering of prednisolone. Symptoms and laboratory and imaging findings gradually improved. Therefore, optic perineuritis due to GPA should be considered a cause of sudden vision loss.