Page 373 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Ophthalmologic and Neuro-Ophthalmologic Aspects of Vasculitides 347

causing conjunctivitis, episcleritis, and keratitis, and optic nerve vasculitis; or as a result of
the contiguous spread of longstanding granulomatous sinusitis leading to proptosis, orbital
pseudotumor and nasolacrimal duct obstruction. Vascular complications such as retinal artery
occlusion can occur [98]. Frequent symptoms of orbital disease included ocular pain,
epiphora and injection although proptosis, vision loss, diplopia and ophthalmoplegia [99,
100]. Rothschild and colleagues [62] noted ocular manifestations in 117 (34%) of 343
patients with GPA, exceeding the expected frequency of 11% for EGPA and 9% for MPA.
Conjunctivitis was noted in 61 (51.2%) patients, followed by episcleritis in 46 (39.3%)
patients, and orbital inflammatory disease in 23 (19.7%) patients. Other ocular manifestations
also included blurred vision in 18 (15.4%) patients, uveitis in 9 (7.7%) patients, oculomotor
nerve palsy in 5 (4.3%) patients, and sudden visual loss in 4 (3.4%) patients. Less common
ocular manifestations were adnexal inflammation, scleritis, blepharitis, keratitis, optic
neuropathy, retinal exudates and hemorrhages.

Hoffman and colleagues [96] noted ocular manifestations of GPA among 15% of 158
patients at presentation, and in 52% of those in the course of the illness. Conjunctivitis and
dacrocystitis each occurred in 20% of patients; however, they were considered nonspecific
features. Painful proptosis, often associated with visual loss due to optic nerve ischemia, and
diplopia resulting from extraocular muscle entrapments, so noted in up to 2% of patients at
onset of disease and in 15% throughout the course of illness, was a useful diagnostic feature
typically caused by retro-orbital pseudotumor. Fauci and co-workers [97] identified proptosis
in 15 of 49 (31%) patients with ocular involvement associated with GPA due to retro-orbital
mass lesions.

Akikusa and co-workers [92] found eye involvement in 13 of 25 (52%) children with
GPA at presentation and in 15 (60%) children over the course of their illness. Similar to
adults, the commonest ocular manifestations were conjunctivitis in 14 (56%) patients, scleritis
or episcleritis in 3 (12%) patients, and proptosis in 2 (8%) patients. Cabral and colleagues
[101] reviewed the presenting clinical features of pediatric patients with GPA in three single-
center cohorts and one multicenter cohort, noting ocular manifestations as common
presenting features of GPA in children, with conjunctivitis occurring in up to 44% at
presentation.

GPA is classified into classic and limited forms, the latter specifically excluding renal
disease with a more favorable prognosis. Although both forms can have ocular manifestations
of disease, Stavrou and colleagues [102] noted sight-threatening manifestations more often in
16 patients with classic GPA than 15 patients with limited GPA, further noting ocular
manifestations in 13 (87%) limited GPA patients compared to 14 (89%) classic GPA patients.
Moreover, 4 (27%) patients in the limited group experienced sight-threatening complications
compared to 8 (50%) patients in the classical group.

Magnetic resonance imaging depicts mucosal inflammation and ulceration in the sinuses,
nasal cavity and orbits in GPA with greater sensitivity than computed tomography (CT) and
can aid in the determination of ocular involvement [103]. The serum ANCA test is useful in
tracking patient responsiveness to systemic therapy. Shiuey and Foster [94] recognized that
patients with GPA and non-normalizing serum ANCA levels after treatment often developed
recurrent ocular despite apparent clinical remission. ANCA serology was further helpful in
determining disease severity and future complications in patients who present with episcleritis
[104], moreover those with positive ANCA serology were more likely to have ocular
complications including keratopathy, visual acuity (VA) less than 20/50, and vascular pannus.

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