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 2. Granulomatosis 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
- usually associated with adjacent paranasal sinus or nasal disease 5
Granulomatosis with polyangiitis is frequently hypointense on T2-weighted images.
- 1. Provenzale JM, Mukherji S, Allen NB, Castillo M, Weber AW. Orbital involvement by Wegener's granulomatosis: imaging findings. AJR. American journal of roentgenology. 166 (4): 929-34. doi:10.2214/ajr.166.4.8610576 - Pubmed
- 2. Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA. Ocular complications of Wegener's granulomatosis. Ophthalmology. 90 (3): 279-90. Pubmed
- 3. STRAATSMA BR. Ocular manifestations of Wegener's granulomatosis. American journal of ophthalmology. 44 (6): 789-99. Pubmed
- 4. J Biswas, K Babu, L Gopal, S Krishnakumar, S Suresh, S Ramakrishnan. Ocular Manifestations of Wegener's Granulomatosis.Analysis of Nine Cases. Indian Journal of Ophthalmology. 51 (3): 217. Pubmed
- 5. Pakalniskis MG, Berg AD, Policeni BA, Gentry LR, Sato Y, Moritani T, Smoker WR. The Many Faces of Granulomatosis With Polyangiitis: A Review of the Head and Neck Imaging Manifestations. AJR. American journal of roentgenology. 205 (6): W619-29. doi:10.2214/AJR.14.13864 - Pubmed