Wegener Granulomatosis-associated Optic Perineuritis

Abstract

The morphological similarity and the anatomical continuity between the meningeal and the perioptic tissues suggest that extension of granulomatous inflammation along such tissue planes accounted for visual loss in these two patients with WG.

Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations

“…Retro-bulbar optic neuritis due to adjacent granulomatosis has been described (51). Takazawa reported optic perineuritis that caused by granulomatous infiltration in two patients (52). Both anterior ischemic optic neuropathy (AION) and posterior ischemic optic neuropathy (PION) have been described in patients with GPA secondary to vasculitis disrupting the blood supply to the optic nerve (31,53).…”

Refractory optic perineuritis due to granulomatosis with polyangiitis successfully treated with methotrexate and mycophenolate mofetil combination therapy

“…In terms of treatment, OPN with GPA responds to high-dose corticosteroid therapy very well, and the ophthalmological prognosis is mostly favorable in the short term. However, patients in whom high-dose corticosteroid treatment was delayed for a long period after the onset of symptoms experienced an irreversible loss of vision (2,4). High-dose corticosteroid therapy should therefore be administered as soon as possible after onset to minimize the possibility of vision loss.…”

“…Yoshitaka Kimura 1,2 , Kurumi Asako 1 After four years of maintenance therapy with PSL and MTX at the dosages indicated above, GPA recurred with an accompanying fever, headache, and exacerbated sinusitis. A chest computed tomography scan showed multiple lung nodules in the upper quadrant of the right lung.…”

“…The predominant phenotype of CNS involvement is hypertrophic pachymeningitis (HP), of which 46% of cases are CNS-GPA (1). Optic perineuritis (OPN), another much less common manifestation of CNS-GPA, is an inflammatory disorder of the optic sheath, which can be diagnosed via magnetic resonance imaging (MRI) by enhancing the optic nerve sheath while sparing the optic nerve parenchyma (2). Herein we report the case of a patient with GPA who presented with HP and refractory OPN, which was manageable only by means of weekly methotrexate (MTX) and mycophenolate mofetil (MMF) combination therapy, but not with MTX, MMF, intravenous cyclophosphamide, rituximab, azathioprine, or cyclosporine individually.…”

Evaluation of Retinal Nerve Fiber Layer in Patients with Idiopathic Optic Perineuritis using Optical Coherence Tomography

“…1,2 In recent, OPN has generally been considered to represent a form of idiopathic inflammatory disease, 2 while OPN occasionally occurs as a manifestation of specific infectious or inflammatory disorders, such as Wegener's granulomatosis, sarcoidosis or syphilis. [3][4][5][6] The prognosis for visual outcome in OPN patients is generally good, but optic nerve atrophy after optic nerve sheath inflammation can occur and worsen the prognosis in some cases of OPN patients. 2 With the increasing use of optical coherence tomography (OCT), many researchers have observed that retinal nerve fiber layer thickness measured by OCT could be a good monitoring technique for quantifying axonal loss in optic neuropathy 7,8 Therefore, OCT findings might be useful diagnostic technique and prognostic indicator in various optic neuropathy.…”