Granulomatosis with polyangiitis (orbital manifestations)

Ophthalmologic manifestations of granulomatosis with polyangiitis (GPA) (previously known as Wegener granulomatosis), both ocular and orbital, have been reported in 40-50% of GPA patients 1-3 and can occur in either the classic or limited form of the disease.4 Ophthalmologic disease occasionally can be the initial clinical manifestation in the limited form of granulomatosis with polyangiitis 2Granulomatosis with polyangiitis is a multi-system systemic necrotizing non-caseating granulomatous vasculitis affecting small to medium-sized arteries, capillaries and veins.

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

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.

  • diffuse inflammatory infiltrate that molds to the orbital contour
  • proptosis
  • usually associated with adjacent paranasal sinus or nasal disease 5

Most orbital pseudotumours are unilateral and either extraconal or transspatial (i.e., both extra- and intraconal) 5.

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

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Article information

rID: 51888
System: Head & Neck
Tag: cases
Synonyms or Alternate Spellings:
  • Wegener granulomatosis (orbital manifestations)
  • Wegener's granulomatosis (orbital manifestations)
  • Orbital granulomatosis with polyangiitis
  • Granulomatosis with polyangiitis with orbital involvement
  • Orbital Wegener's granulomatosis

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