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.