Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (irvan) syndrome associated with positive perinuclear antineutrophil cytoplasmic antibody (p-anca).

PURPOSE To describe an unusual case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome associated with a positive perinuclear antineutrophil cytoplasmic antibody test. METHODS This is an interventional case report. A 22-year-old man with decreased vision underwent ophthalmoscopic, angiographic, and laboratory evaluation. The left eye underwent laser therapy due to circinate retinopathy and more severe involvement. RESULTS Extensive systemic workup was negative. The only positive laboratory test was a positive perinuclear antineutrophil cytoplasmic antibody (1/40 dilution) detected by indirect immunofluorescence and enzyme-linked immunosorbent assay (positive for antimyeloperoxidase antibody). The patient was observed for 2 years. The disorder remained limited to the eyes, and further systemic workup was unrevealing; perinuclear antineutrophil cytoplasmic antibody titer remained positive. Vision remained stable in the treated eye and was slightly reduced in the untreated eye. CONCLUSION Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome may be suggestive of a retinal form of perinuclear antineutrophil cytoplasmic antibody-associated vasculitis.