Page 375 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Ophthalmologic and Neuro-Ophthalmologic Aspects of Vasculitides 349

inflammatory and ischemic vasculitis types. Patients with the pseudotumor type typically
present with a chronically red eye, dacryoadenitis, myositis, periscleritis, perineuritis,
conjunctival granuloma, episcleritis, orbital abnormalities on imaging, and ANCA
seronegativity. Those with the ischemic vasculitis present with sudden visual loss, amaurosis
fugax, anterior ischemic optic neuropathy, central retinal artery or branch retinal artery
occlusion, normal orbital imaging, and ANCA seropositivity.

Among 270 patients with EGPA [62] 30 (11%) patients had ocular manifestations of
which conjunctivitis was the most common so noted in 13 (43.3%) patients followed by
blurred vision in 9 (30%) patients, oculomotor nerve palsy and sudden visual loss each in 4
(13.3%) patients. retinal vasculitis in 3 (12%) patients; orbital inflammatory disease, and
retinal exudates each in 2 (6.7%) patients; and episcleritis, keratitis, uveitis, retinal
thrombosis, and retinal exudates present each in 1 (3.3%) patient. Androudi and colleagues
[114] described a patient with a 20 year history of severe steroid-dependent asthma,
hematuria, pleuritis and sinusitis, who later developed uveitis and bilateral scleritis. Anterior
ischemic optic neuropathy was reported by Lee and colleagues [115] in a 54- year-old man
with bronchial asthma, allergic rhinitis, and sinusitis who presented with a sudden decrease in
VA. Central retinal artery occlusion, another complication of EGPA, leading to sudden visual
loss, was described among several other patients. Skrapari and colleagues [116] described a
50-year-old woman with left foot drop and painless visual loss in whom fundoscopy revealed
central retinal artery occlusion. Kumano and co-workers [117] reported a 54-year-old woman
with sudden loss of vision after tapering prednisolone from 30 mg to 10 mg per day, in whom
central retinal artery occlusion was noted on FFA that showed delayed filling of the retinal
and cilioretinal arteries, without leakage secondary to vasculitis. Hamann and Johansen [118]
reported severe visual loss in association with central retinal artery occlusion evidenced by
retinal whitening, macular cherry-red spot, combined with central retinal vein occlusion so
noted by papilledema, retinal hemorrhages, and dilated and tortuous veins on Fundoscopy;
fluorescein angiography revealed an absence of retinal filling.

Among 96 patients with EGPA described by Guillevin and colleagues [110] 3 (3.1%)
patients presented with ophthalmic involvement. 2 had episcleritis, and 1 had bilateral
exophthalmos. Koenig and co-workers [119] reported a patient with monocular blindness due
to central retinal artery occlusion as the presenting feature of EGPA. McNab [120] described
a 66-year-old woman with diplopia, foreign body sensation, redness and proptosis of the left
eye, in whom orbital CT scan revealing increased density of the left orbital fat. In addition,
Jordan and co-workers [121] reported two cases of EGPA who presented with dacryoadenitis
and diffuse orbital inflammation. Takanashi and colleagues [113] recommended enhanced
orbital imaging to look for inflammatory lesions. Fundoscopy is useful in identifying patients
with central retinal artery occlusion.

The treatment of EGPA typically consists of corticosteroids and cyclophosphamide
[122]; however plasma exchange can be added to treat patients with advanced renal disease,
with expected survival rates approaching 90% [123]. There are no randomized, controlled
studies of the efficacy of treatment on the ocular manifestations of EGPA. McNab [120]
described a 66-year-old woman with orbital inflammation who responded rapidly and
completely to an oral prednisolone taper starting at 50 mg. A patient with anterior ischemic
optic neuropathy responded to treatment with 24 mg of methylprednisolone daily however
after one month, VA improved from 20/50 to 20/25 in addition to improved respiratory
symptoms. Followup fundoscopy revealed subsiding papilledema and splinter hemorrhages

Complimentary Contributor Copy