Granulomatosis with polyangiitis (orbital manifestations)

Ophthalmologic manifestations in granulomatosis with polyangiitis , including both ocular and orbital diseases, have been reported in 40-50% of GPA patients 1,2,3 and can occur in either the classic or the limited form of the disease.4

Ophthalmologic disease occasionally can be the initial clinical manifestation in the limited form of GPA.2

Ophthalmologic involvement include:

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

Orbital granulomas can be characterized as contiguous (secondary to extension of granulomatous disease from the paranasal sinuses) or 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 of the intracavernous sinus may manifest as Tolosa-Hunt syndrome with painful opthalmoplegia.5

CT

Diffuse inflammatory infiltrate that molds to the contour of the orbit, proptosis, usually associated with adjacent paranasal sinus or nasal disease.5

Most orbital pseudotumors are unilateral and either extraconal or transspatial (both extra- and intraconal).5

MRI

Wegener granulomatosis is frequently hypointense on T2-weighted images.

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Article Information

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

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