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Granulomatosis with polyangiitis (GPA) is a multisystemic, antineutrophil cytoplasmic antibody (ANCA)–associated small vessel vasculitis. The orbit and ocular tissues are implicated in approximately a third of cases.
In this report we present a characteristic imaging finding in orbital GPA with postcontrast pachymeningeal enhancement associated with orbital inflammation on T1-weighted, fat-suppressed magnetic resonance imaging (MRI) imaging.
A 42-year-old man was referred to the ophthalmology department with a 1-month history of bilateral watery, red eyes; diplopia; and headache. The presentation was on the background of a 7-month history of a lower back ulcer being managed as pyoderma gangrenosum with prednisolone and cyclosporine.
Visual acuities were 6/9 right and 6/6 left. There was a right relative afferent pupillary defect and impaired colour vision as measured with Ishihara plates (4 out of 13). He had bilateral proptosis of 22 mm associated with upper and lower lid swelling (Fig. 1). Movement of the right eye was limited in all directions of gaze with accompanying diplopia except on downgaze. There was bilateral conjunctival injection, and fundus examination demonstrated choroidal folds in the right eye.
MRI demonstrated enhancement of the pachymeninges of the frontal lobes bilaterally, middle cranial fossa, anterior falx cerebri, and left parietal lobe. T1-weighted, postcontrast, fat-suppressed MRI demonstrated bilateral, inferior extraconal enhancing infiltrates in the orbits involving the periosteum (Fig. 2). The mass in the right orbit appeared to extend to the orbital apex. Additionally there was mild thickening of the inferior recti bilaterally.
Cytoplasmic ANCA was positive with a proteinase 3 titre of 80 IU/mL, which in conjunction with the clinical and radiological findings was highly suspicious of GPA. He was admitted to hospital with a view to undergo orbital biopsy but developed central nervous system–associated vasculitis and so was treated urgently with pulsed 1 g intravenous (IV) methylprednisolone for 3 days and 1 g rituximab. Despite treatment he further deteriorated soon after, with increasing proptosis and loss of vision in his right eye to perception of light only, and so was treated again with a further 3-day pulse of 1 g IV methylprednisolone followed by 500 mg IV cyclophosphamide. He then received a further dose of 1 g IV rituximab as well as 5 further fortnightly doses of 500 mg IV cyclophosphamide in addition to a tapering dose of oral prednisolone. At the last review, vision in the right had improved to 6/21 (6/18 with pinhole) with an associated dense, inferior altitudinal visual field defect. He remains under the care of the immunology team with a current immunosuppressive regime of mycophenolate 1000 mg twice daily and prednisolone 5 mg daily.
Orbital inflammatory disease shares several etiologies with pachymeningitis such as sarcoidosis and IgG4-related disease. The presence of both concurrently is unusual and is highly suggestive of GPA.
Imaging findings typical of orbital GPA include obliteration and infiltration of fat planes and erosion of bone. The theory behind these findings is of contiguous spread of inflammation from the paranasal sinuses to the orbit. It has been suggested that central nervous system involvement, as seen in this case, is also a consequence of the same mechanism.
Pachymeningeal involvement was typical of refractory disease and poorer prognosis in this series. In a retrospective series of 6 patients with orbital inflammation and pachymeningitis, 4 had GPA and 2 had tuberculosis.