Page 374 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

348 Sarah Conway and David S. Younger

Fauci and colleagues [97] recommended remission induction treatment of GPA with
2mg/kg/day of oral cyclophosphamide and 1mg/kg/day of prednisone, followed by tapering
of prednisone to an alternate day administration, achieving complete remission rates of 93%
for a mean duration of 48.2 months. Chan and colleagues [98] reported a patient with bilateral
corneal ulcers and a VA reduced to 20/200 in the right eye and 20/70 in the left eye that was
treated with 2 mg/kg/day of oral cyclophosphamide and 1mg/kg/d of prednisone. Two years
later the VA in both eyes returned to 20/40, and the peripheral corneal ulcers healed although
shallow peripheral corneal thinning remained. Foster and colleagues [105] studied a patient
with GPA and progressive blurred vision, diplopia and increased supraorbital pressure, who
was found to have a VA of 20/200 in the right eye, 20/40 in the left eye, 90%
ophthalmoplegia in the left eye, and normal fundoscopic exam. The patient was treated with
oral cyclophosphamide and prednisone, and one month later VA improved to 20/40 with
complete resolution of ophthalmoplegia. Vischio and McCrary [106] who reported a 70-year-
old man with left eye visual change, third nerve palsy and orbital mass compressing the optic
nerve that was biopsied with proven GPA. The patient was treated with prednisone 40mg
twice daily and cyclophosphamide 125mg daily, without visual improvement eleven weeks
later. There is substantial ocular morbidity associated with GPA despite efficacious
immunosuppressant therapy so noted in three enucleations among 140 patients [95]. Sadiq
and colleagues [100] identified a subgroup of patients with GPA and orbital involvement with
a poor prognosis evidenced by permanent visual loss in 43% of the patients.

Some patients with episcleritis, conjunctivitis, and anterior uveitis respond to topical
therapy, but they should not be used because of secondary corneal thinning and perforation
[107]. Although the surgical intervention is generally reserved for the performance of tissue
biopsies, dacryocystorhinistomy was effective therapy for nasolacrimal duct obstruction,
achieving symptomatic relief in 13 of 14 (93%) of patients [108].

Eosinophilic Granulomatosis with Polyangiitis

This systemic necrotizing small vessel vasculitis involves multiple organ systems,
typically causing chronic rhinosinusitis, asthma, and eosinophilia. It is a rare disorder with an
estimated annual incidence of 2.7 cases per 1,000,000 people and a period prevalence rate of
6.8 per million in those taking anti-asthma drugs [89, 109]. With most patients developing the
disease in the fourth decade of life between age 14 and 74 years, EGPA has no gender
predominance [62, 88, 110].

The ACR 1990 diagnostic criteria for EGPA [111] included six criteria, four of which
were necessary from the following including, asthma, eosinophilia greater than 10%,
neuropathy, pulmonary infiltrates, paranasal sinus abnormality, and extravascular eosinophils.
The presence of four or more criteria yielded a sensitivity of 85% and a specificity of 99.7%
for the diagnosis of EGPA. Although ANCA positivity is not part of the ACR?s diagnostic
criteria, it can be helpful in making the diagnosis in up to 70% of patients [88]. The
characteristic changes of EGPA histopathology include necrotizing vasculitis and extra-
vascular necrotizing granulomas with eosinophilic infiltrates; however, early cases may be
characterized by tissue infiltration by eosinophils without overt vasculitis [112].

Ocular features of EGPA can involve all parts of the eye and orbit. Takanashi and co-
workers [113] classified the ocular manifestations into two types, pseudotumor or orbital

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