Granulomatosis with polyangiitis (orbital manifestations)

Last revised by Bruno Di Muzio on 29 Aug 2020

Ophthalmologic manifestations of granulomatosis with polyangiitis (GPA) is not uncommon and can occur in either the classic or limited form of the disease.

For a general discussion of the condition, please refer to the main article on granulomatosis with polyangiitis (GPA). For other organ-specific radiographic features, please refer to individual articles:

Granulomatosis with polyangiitis (previously known as Wegener granulomatosis), is a multi-system systemic necrotizing non-caseating granulomatous vasculitis affecting small to medium-sized arteries, capillaries and veins.

Both ocular and orbital involvement in GPA has been reported in 40-50% of patients 1-3. Ophthalmologic disease occasionally can be the initial clinical manifestation in the limited form of granulomatosis with polyangiitis 2

Ophthalmologic involvement includes:

  • granulomatous disease causing an orbital inflammatory mass, often with proptosis and/or optic nerve compression
  • small-vessel vasculitis causing conjunctivitis, scleritis, episcleritis, uveitis, optic neuritis, optic nerve vasculitis, or retinitis 2,4

Orbital granulomas can be characterized as

  • contiguous: extension of granulomatous disease of the paranasal sinuses
  • focal: arising primarily within the orbit 3
  • diffuse inflammatory infiltrate that molds to the orbital contour
  • proptosis
  • usually associated with adjacent paranasal sinus or nasal disease 5

Most orbital pseudotumors are unilateral and either extraconal or trans spatial (i.e., both extra- and intraconal) 5.

Granulomatosis with polyangiitis is frequently hypointense on T2-weighted images.

Complications include optic nerve compression with subsequent atrophy and visual loss. Extension of the disease to the cranial nerves traversing the cavernous sinus may manifest as Tolosa-Hunt syndrome with painful ophthalmoplegia 5.

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