Optic neuritis in a teenage girl with granulomatosis with polyangiitis

Dejan Bokonjić; Nada Avram; Predrag Minić; Aleksandra Radosavljević

doi:10.2298/VSP190223061B

Dejan Bokonjić University of East Sarajevo, Faculty of Medicine, Foča, Republic of Srpska, Bosnia and Herzegovina
Nada Avram University of East Sarajevo, Faculty of Medicine, Foča, Republic of Srpska, Bosnia and Herzegovina
Predrag Minić Mother and Child Health Care Institute of Serbia “Dr. Vukan Čupić“, Belgrade, Serbia
Aleksandra Radosavljević University of Belgrade, Faculty of Medicine, Belgrade, Serbia

DOI: https://doi.org/10.2298/VSP190223061B

Keywords: diagnosis, optic nerve, optic neuritis, treatment outcome, wegener granulomatosis

Abstract

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 the respiratory tract and kidneys. Clinical manifestations can be diverse, 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 extrapulmonary 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 ophthalmologist 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 segment findings. On ophthalmoscopy, the left optic nerve oedema 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 confirmed the diagnosis of optic perineuritis. After pulse doses of methylprednisolone, the girl achieved complete resolution of vision in the left eye. Conclusion. If untreated, inflammation of the optic nerve can lead to a permanent loss of vision. Prompt diagnostic and adequate treatment of patients with GPA is needed in order to prevent vision-threatening complications and control the systemic disease.

Author Biography

Predrag Minić, Mother and Child Health Care Institute of Serbia “Dr. Vukan Čupić“, Belgrade, Serbia

None

References

Grygiel-Górniak B, Limphaibool N, Perkowska K, Puszczewicz M. Clinical manifestations of granulomatosis with polyangiitis: key considerations and major features. Postgrad Med 2018; 130(7): 581‒96.

Lamprecht P, Kerstein A, Klapa S, Schinke S, Karsten CM, Yu X, et al. Pathogenetic and clinical aspects of anti-neutrophil cy-toplasmic autoantibody-associated vasculitides. Front Immu-nol 2018; 9: 680.

Jariwala MP, Laxer RM. Primary vasculitis in childhood: GPA and MPA in childhood. Front Pediatr 2018; 6(8): 226.

Bohm M, Gonzalez Fernandez MI, Ozen S, Pistorio A, Dolezalova P, Brogan P, et al. Clinical features of childhood granulomato-sis with polyangiitis (wegener's granulomatosis). Pediatr Rheumatol Online J 2014; 12: 18.

Tarabishy AB, Schulte M, Papaliodis GN, Hoffman GS. Wegen-er’s granulomatosis: Clinical manifestations, differential diag-nosis, and management of ocular and systemic disease. Surv Ophthalmol 2010; 55(5): 429‒44.

Rothschild PR, Pagnoux C, Seror R, Brézin AP, Delair E, Guillevin L. Ophthalmologic Manifestations of Systemic Necrotizing Vasculitides at Diagnosis: A Retrospective Study of 1286 Pa-tients and Review of the Literature. Semin Arthritis Rheum 2013; 42(5): 507‒14.

Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 crite-ria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990; 33(8): 1101‒7.

Thai LH, Charles P, Resche-Rigon M, Desseaux K, Guillevin L. Are anti-proteinase-3 ANCA a useful marker of granulomato-sis with polyangiitis (Wegener's) relapses? Results of a retro-spective study on 126 patients. Autoimmun Rev 2014; 13(3): 313‒8.

Pagnoux C, Guillevin L. Treatment of granulomatosis with pol-yangiitis (Wegener's). Expert Rev Clin Immunol 2015; 11(3): 339‒48.

Shafiei K, Luther E, Archie M, Gulick J, Fowler MR. Wegener granulomatosis: case report and brief literature review. J Am Board Fam Pract 2003; 16(6): 555‒9.

Pakrou N, Selva D, Leibovitch I. Wegener's granulomatosis: oph-thalmic manifestations and management. Semin Arthritis Rheum 2006; 35(5): 284‒92.

Kubaisi B, Abu Samra K, Foster CS. Granulomatosis with pol-yangiitis (Wegener's disease): An updated review of ocular dis-ease manifestations. Intractable Rare Dis Res 2016; 5(2): 61‒9.

Ismailova DS, Abramova JV, Novikov PI, Grusha YO. Clinical features of different orbital manifestations of granulomatosis with polyangiitis. Graefes Arch Clin Exp Ophthalmol 2018; 256(9): 1751‒6.

Paović J, Paović P, Vukosavljević M. Clinical and immunological features of retinal vasculitis in systemic diseases. Vojnosanit Pregl 2009; 66(12): 961‒5.

Purvin V, Kawasaki A. Optic perineuritis secondary to Wegen-er's granulomatosis. Clin Exp Ophthalmol 2009; 37(7): 712‒7.

Takazawa T, Ikeda K, Nagaoka T, Hirayama T, Yamamoto T, Yanagihashi M, et al. Wegener granulomatosis-associated optic perineuritis. Orbit 2014; 33(1): 13‒6.

Shunmugam M, Morley AM, Graham E, D'Cruz D, O'Sullivan E, Malhotra R. Primary Wegener's granulomatosis of the orbital apex with initial optic nerve infiltration. Orbit 2011; 30(1): 24‒6.

Aakalu VK, Ahmad AZ. Wegener granulomatosis causing compressive optic neuropathy in a child. Ophthalmic Plast Reconstr Surg 2009; 25(4): 327‒8.