Granulomatosis with polyangiitis (orbital manifestations)

Ophthalmologic manifestations of granulomatosis with polyangiitis (GPA) (previously known as Wegener's 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.2 Granulomatosis with polyangiitis (Wegener granulomatosis), 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 opthalmoplegia.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:
  • Orbital Wegener granulomatosis
  • Orbital granulomatosis with polyangiitis
  • Granulomatosis with polyangiitis with orbital involvement
  • Wegener granulomatosis (orbital manifestations)
  • Wegener's granulomatosis (orbital manifestations)

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