Page 371 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Ophthalmologic and Neuro-Ophthalmologic Aspects of Vasculitides 345

Kawasaki Disease

This medium vessel vasculitis is one of commonest vasculitides in children with
incidence rates varying from 8.39 per 100,000 children under age 5 per year in England [69]
to 218.6 per 100,000 children under age 5 in Japan [70]. The disease occurs more often in
males, with a male to female ratio of 1.3-1.6:1 [71, 72]. KD typically occurs in patients under
5 years of age, with a peak age of onset ranging from 9 to 11 months in Japan [70], and over
12 months in Canada and Britain [73, 74]. There are six diagnostic features of KD, five of
which are needed for the diagnosis. They include fever of unknown origin lasting five days or
more not responding to antibiotics; bilateral conjunctival hyperemia and indurated edema that
spares the limbal region; orolabial lesions; redness and edema of palms and soles followed by
fingertip desquamation; an erythematous polymorphous rash; and cervical lymph node
enlargement [71].

Ocular involvement in KD is typified by bilateral conjunctival hyperemia and indurated
edema that spares the limbal region; and conjunctivitis that occurs in 83% to 92% of patients
[74]. The conjunctival lesion typically develops within a day or two of fever-onset and lasts
up to several months [75]. Other ocular findings included bilateral injection of the bulbar
conjunctiva so noted in up to 89% of patients, bilateral iridocyclitis in 78% of patients,
superficial punctate keratitis in 22%, vitreous opacities and papilledema each in 11%, and
subconjunctival hemorrhage in 6% of patients. Bulbar conjunctival injection typically occurs
first, followed by acute iridocyclitis identified by inflammatory cells in the anterior chamber
that reaches a maximum between the fifth and eighth day, thereafter diminishing after one
month. Acute bilateral iridocyclitis is more common in children 4 years and older however
the severity of iridocyclitis is generally independent of age [76]. Burke and co-workers [77]
noted that anterior uveitis was a predominant finding during the acute phase of illness. Other
less common ocular findings in KD include disciform keratitis, so noted in a 10-year-old [78]
and 11-year-old reported children [79], the latter of who presented with diffuse bilateral non-
purulent conjunctival congestion, VA of 6/6 in the right eye and 6/5 in the left eye, and later
developed cloudy vision. Ophthalmologic examination revealed that his vision was reduced to
less than 6/60 in both eyes and that he had bilateral central stroma edema with localized
keratitic precipitates. However, fundoscopy showed bilateral disc swelling without evidence
of posterior segment inflammation. The latter, also rare, was similarly documented in several
patients, including.

Posterior segment involvement in KD was documented in two patients with bilateral
vitreous opacities and bilateral optic disc swelling [76], unilateral retinal exudates, macular
and disc edema with severe visual loss [80] and in a patient with bilateral inner retinal
ischemia diagnosed at postmortem examination [81]. Retinal vasculitis occurs rarely in KD
due to selective inflammation of the blood-ocular barrier [76]. Several reported patients
underwent FFA for retinal vasculitis, including one with retinal exudation, macular edema,
and temporal disc swelling that showed no leakage [82], and another with bilateral acute
anterior uveitis, in whom FFA showed disc edema and leakage with localized areas of
perivascular sheathing suggestive of periphlebitis and vasculitis [83].

First line therapy for KD includes intravenous immune globulin (IVIg) therapy and
aspirin. However corticosteroids and tumor necrosis factor (TNF) inhibitors may beneficial
those who fail to respond to IVIg therapy [84, 85]. A few small series and case reports
showed a favorable response of conjunctivitis and anterior uveitis respond to treatment with

Complimentary Contributor Copy