Granulomatosis with polyangiitis (orbital manifestations)

Granulomatosis
with polyangiitis (orbital manifestations) • Granulomatosis with polyangiitis - orbital involvment - Ganzer Fall bei Radiopaedia
Ibrahim, D. Granulomatosis with polyangiitis - orbital involvment. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-51884 • CC by-nc-sa 3.0 (modified)
Granulomatosis
with polyangiitis • Granulomatosis with polyangiitis - orbital involvment - Ganzer Fall bei Radiopaedia
Ibrahim, D. Granulomatosis with polyangiitis - orbital involvment. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-51884 • CC by-nc-sa 3.0 (modified)
Radiopaedia • CC-by-nc-sa 3.0 • de

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.

Epidemiology

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

Clinical presentation 

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 

Pathology

Orbital granulomas can be characterized as

  • contiguous: extension of granulomatous disease of the paranasal sinuses
  • focal: arising primarily within the orbit

Radiographic features

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

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

MRI

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

Treatment and prognosis

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 .

Differential diagnosis