Optic neuritis in a teen-age girl with granulomatosis with polyangiitis

Dejan Bokonjic, Nada Avram, Predrag Minic, Aleksandra Radosavljevic
2019 Vojnosanitetski Pregled  
Introduction. Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is characterized by necrotizing granulomatous inflammation in various tissues, including blood vessels, but primarily in respiratory tract and kidneys. Clinical manifestations can be diverse, including inflammation of the eye and adnexa. Disease can manifest at all ages, but is rare in children. Case report. In this report, we present a case of 16 years old girl with rare extra-pulmonary
more » ... ulmonary manifestation of GPA. Girl had previous history of GPA and complained of a sudden blurred vision in the left eye. She was promptly referred to the ophthalmologist who noted 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 segment findings. On ophthalmoscopy, left optic nerve oedema was noted. Urgent CT of left orbit showed a soft tissue mass around the optic nerve in the apex of the orbit. Magnetic resonance imaging confirmed the diagnosis of optic perineuritis. After pulse doses of methylprednisolone girl achieved complete resolution of vision in the left eye. Conclusion. Optic neuritis is a very rare ophthalmological manifestation of GPA, not previously described in a teenager. All previous reports included only elder patients in seventh or eighth decade of life. If untreated, inflammation of the optic nerve can lead to permanent loss of vision. Prompt diagnostic and adequate treatment are needed in order to prevent vision threatening complications and to control the systemic disease. Key words: granulomatosis with polyangiitis, optic perineuritis, medicamentous treatment. Apstrakt Uvod. Granulomatoza sa poliangiitisom (GPA), ranije poznata kao Wegener-ova granulomatoza, se karakteriše nekrotizujućom granulomatoznom inflamacijom u brojnim tkivima, uključujući krvne sudove, ali primarno unutar respiratornog trakta i bubrega. Kliničke manifestacije mogu biti raznovrsne uključujući i zapaljenje oka i adneksa. Bolest se može manifestovati u bilo kom uzrastu, ali je retka kod dece. Prikaz slučaja. U ovom izveštaju prikazan je slučaj šesnaestogodišnje devojčice sa retkom ekstra-pulmonalnom manifestacijom GPA. Devojčica, ranije lečena zbog GPA, se požalila na iznenadni pad vida na levom oku. Hitno je upućena oftalmologu koji je ustanovio smanjenu vidnu oštrinu 4 levog oka 3/60. Kolorni vid je bio oštećen u spektru crvene boje. Kliničkim pregledom utvrđen je normalan nalaz na prednjem segmentu oka. Pregledom očnog dna uočen je edem vidnog živca levo. Hitan CT leve orbite je pokazao mekotkivnu masu oko optikusa u vrhu orbite. Magnetna resonanca je potrvrdila dijagnozu optičkog perineuritisa. Nakon pulsnih doza metilprednizolona došlo je do potpunog oporavka vidne funkcije levog oka. Zaključak. Optički neuritis je veoma retka oftalmološka manifestacija GPA, koja do sada nije opisana kod mladih osoba. Svi raniji opisani slučajevi su bile osobe u 7. ili 8. deceniji života. Ukoliko se zapaljenje optikusa ne leči, može dovesti do trajnog gubitka vida. Stoga je neophodno hitno sprovesti kompletnu dijagnostiku i adekvatno lečenje, kako bi se sprečile po vid preteće komplikacije i kako bi se kontrolisala sistemska bolest. Ključne reči: granulomatoza sa poliangiitison, optički perineuritis, medikamentno lečenje.
doi:10.2298/vsp190223061b fatcat:m2dnnfcdkba6dhnqg5imbffdpu