Bokonjic, Dejan (6701490505)Dejan (6701490505)BokonjicAvram, Nada (57223127632)Nada (57223127632)AvramMinic, Predrag (6603400160)Predrag (6603400160)MinicRadosavljevic, Aleksandra (56993158000)Aleksandra (56993158000)Radosavljevic2025-06-122025-06-122021https://doi.org/10.2298/VSP190223061Bhttps://www.scopus.com/inward/record.uri?eid=2-s2.0-85104954106&doi=10.2298%2fVSP190223061B&partnerID=40&md5=1abf9b8f7368339e4f31b5e3b1580781https://remedy.med.bg.ac.rs/handle/123456789/4479Introduction. Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is character-ized by necrotizing granulomatous inflammation in various tissues, including blood vessels, but primarily in the respira-tory tract and kidneys. Clinical manifestations can be di-verse, including inflammation of the eye and adnexa. Optic neuritis is a very rare ophthalmological manifestation of GPA, not previously described in a teenager. Case report. We presented a case of a 16-year-old girl with a rare extrap-ulmonary manifestation of GPA. The girl had a previous history of GPA and complained of a sudden blurred vision in the left eye. She was promptly referred to an ophthalmol-ogist who noted a decreased visual acuity of 20/400 in the left eye. Colour vision was impaired in the spectrum of red colour. Clinical examination revealed normal anterior seg-ment findings. On ophthalmoscopy, the left optic nerve oe-dema was noted. Urgent computed tomography of the left orbit showed a soft tissue mass around the optic nerve in the apex of the orbit. Magnetic resonance imaging con-firmed the diagnosis of optic perineuritis. After pulse doses of methylprednisolone, the girl achieved complete resolu-tion of vision in the left eye. Conclusion. If untreated, in-flammation of the optic nerve can lead to a permanent loss of vision. Prompt diagnostic and adequate treatment of pa-tients with GPA is needed in order to prevent vision-threatening complications and control the systemic disease. © 2021 Inst. Sci. inf., Univ. Defence in Belgrade. All rights reserved.DiagnosisOptic nerveOptic neuritisTreatment outcomeWegener granulomatosis