Advertisement

Pachymeningeal enhancement on magnetic resonance imaging in granulomatosis with polyangiitis

      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.
      • Rothschild PR
      • Pagnoux C
      • Seror R
      • Brezin AP
      • Delair E
      • Guillevin L
      Ophthalmologic manifestations of systemic necrotizing vasculitides at diagnosis: a retrospective study of 1286 patients and review of the literature.
      Common manifestations are conjunctivitis, episcleritis, orbital inflammation, and uveitis.
      • Rothschild PR
      • Pagnoux C
      • Seror R
      • Brezin AP
      • Delair E
      • Guillevin L
      Ophthalmologic manifestations of systemic necrotizing vasculitides at diagnosis: a retrospective study of 1286 patients and review of the literature.
      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.
      Fig 1
      Fig. 1Photograph on presentation demonstrating proptosis, chemosis conjunctival injection, and lid swelling.
      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.
      Fig 2
      Fig. 2Coronal T1-weighted, fat-suppressed magnetic resonance imaging scan demonstrating enhancement of the pachymeninges and falx cerebri and inferior extraconal enhancing infiltrates in both orbits involving the periosteum.
      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.
      • Cannon PS
      • Cruz AA
      • Pinto CT
      • et al.
      A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation.
      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.
      • Cannon PS
      • Cruz AA
      • Pinto CT
      • et al.
      A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation.
      ,
      • Seror R
      • Mahr A
      • Ramanoelina J
      • Pagnoux C
      • Cohen P
      • Guillevin L
      Central nervous system involvement in wegener granulomatosis.
      GPA is the most commonly reported specific diagnosis in the literature on orbital inflammation associated with pachymeningeal enhancement.
      • Cannon PS
      • Cruz AA
      • Pinto CT
      • et al.
      A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation.
      In a study of 59 patients with orbital ANCA-associated vasculitis, 56 of whom had GPA, 12 were found to have contiguous pachymeningitis.
      • Durel CA
      • Hot A
      • Trefond L
      • et al.
      Orbital mass in anca-associated vasculitides: data on clinical, biological, radiological and histological presentation, therapeutic management, and outcome from 59 patients.
      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.
      • Cannon PS
      • Cruz AA
      • Pinto CT
      • et al.
      A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation.
      The mechanism for periorbital postcontrast enhancement on MRI may be secondary to development of periostitis from granulomatous and vasculitic involvement of the sinus mucosa and periosteum.
      • Seror R
      • Mahr A
      • Ramanoelina J
      • Pagnoux C
      • Cohen P
      • Guillevin L
      Central nervous system involvement in wegener granulomatosis.
      Long bone biopsies in patients with GPA have demonstrated periostitis and vasculitis and correlate with new bone growth on x-ray.
      • Hamidou MA
      • Dupas B
      • Moreau A
      Periosteal new bone formation in wegener’s granulomatosis.
      Clinicians should be aware of this imaging finding typifying GPA when evaluating orbital inflammation, particularly when thickening and inflammation of the periorbita is documented.

      Footnotes and Disclosure

      The authors have no proprietary or commercial interest in any materials discussed in this article.

      References

        • Rothschild PR
        • Pagnoux C
        • Seror R
        • Brezin AP
        • Delair E
        • Guillevin L
        Ophthalmologic manifestations of systemic necrotizing vasculitides at diagnosis: a retrospective study of 1286 patients and review of the literature.
        Semin Arthritis Rheum. 2013; 42: 507-514
        • Cannon PS
        • Cruz AA
        • Pinto CT
        • et al.
        A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation.
        Orbit. 2011; 30: 64-69
        • Seror R
        • Mahr A
        • Ramanoelina J
        • Pagnoux C
        • Cohen P
        • Guillevin L
        Central nervous system involvement in wegener granulomatosis.
        Medicine. 2006; 85: 54-65
        • Durel CA
        • Hot A
        • Trefond L
        • et al.
        Orbital mass in anca-associated vasculitides: data on clinical, biological, radiological and histological presentation, therapeutic management, and outcome from 59 patients.
        Rheumatology. 2019; 58: 1565-1573
        • Hamidou MA
        • Dupas B
        • Moreau A
        Periosteal new bone formation in wegener’s granulomatosis.
        J Rheumatol. 1997; 24: 814-815