Page 372 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

346 Sarah Conway and David S. Younger

corticosteroids and mydriatics [80], IVIg therapy and aspirin [83]. One other patient with
disciform keratitis and bilateral disc swelling, without signs of posterior segment
inflammation, treated with topical steroids every 2 hours, cyclopentolate 1% twice a day and
oral acyclovir 200 mg five times a day, experienced rapid resolution of ocular signs and
symptoms. His VA improved from 6/60 bilaterally to his baseline of 6/6 in the right eye and
6/5 in the left eye after three weeks of therapy [86]. However, since anterior uveitis can be
self-limiting in 2 to 8 weeks, it can be difficult at times to ascertain the true impact of
treatment on symptom resolution. Rennebohm [87] described six children with KD, five of
whom developed anterior uveitis during the acute phase of KD. Both children treated with
corticosteroids and cycloplegic drugs improved, as did the other three untreated children.
Similarly, Puglise [75] described a 4-year-old child with KD who presented with bilateral
swelling and hyperemia of the conjunctiva unresponsive to intense topical steroid therapy;
however there was a reduction in conjunctival inflammation within one week of 30mg/kg of
aspirin therapy and complete resolution in four weeks.

Small Vessel Vasculitis

Anca-Associated

Granulomatosis with Polyangiitis
This systemic necrotizing SVV is characterized by granulomatous inflammation of the

upper and lower respiratory tract, with focal necrotizing glomerulonephritis. The disease
shows a strong predominance for Caucasians, particularly those of northern European
ancestry with a prevalence ranging from 23.7 cases per 1,000,000 people in France [88], to 63
cases per 1,000,000 people in the United Kingdom [89], and 95 cases per 1,000,000 people in
Northern Norway [90]. It is slightly more common in men than in women with a 1.3 to 1.7:1
male to female ratio [62, 91], and typically occurs in the fourth and fifth decades of life [62,
88, 91]. The disease is rare in children, but when it occurs, it is four times more common in
girls than boys [92]. The histopathology of GPA is characterized by a triad of multinucleated
giant cell granulomatous inflammation, vasculitis, and necrosis [93].

The ACR [91] identified four diagnostic criteria for GPA, two of which must be present
in order to make the diagnosis from among the following including, nasal or oral
inflammation, an abnormal chest radiographic showing nodules, fixed infiltrates, or cavities,
urinary sediment showing microhematuria or red cell casts, and granulomatous inflammation
on biopsy. The presence of two or more criteria imparted a sensitivity of 88.2% and
specificity of 92.0% for the diagnosis of GPA. Although serum ANCA level is not part of the
ACR criteria, GPA is associated with ANCA, particularly c-ANCA, the presence of which
had 99% specificity and 96% sensitivity for generalized GPA, and 67% sensitivity for the
limited form [94]. Ocular findings are not part of the diagnostic criteria for GPA; however
among patients with vasculitis [91], ocular inflammation, so noted as scleritis, episcleritis,
and proptosis, had a sensitivity of 27.4% and a specificity of 96.9% for the diagnosis of GPA.

Ocular manifestations occur overall in 30% to 60% of patients with GPA [95-97], and are
the presenting features in up to 16% of patients [97]. Ocular symptoms can due to primary
inflammation or focal vasculitis that affects the anterior and posterior segments of the eye

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