Page 381 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Ophthalmologic and Neuro-Ophthalmologic Aspects of Vasculitides 355

from counting fingers to 6/36 with 4 mg of daily prednisolone. Cystoid macular edema and
cotton wool spots were noted to respond to 60 mg of oral prednisone with resolution of the
macular edema in two days [170].

Variable Vessel Vasculitis

Behçet Disease

This disorder is characterized by relapsing aphthous ulcers of the mouth, eye and
genitalia [172]. The most widely used diagnostic criteria of BD were formulated by the
International Study Group (ISG) [173] that include recurrent oral ulcerations plus any two of
genital ulceration, typical defined eye lesions, typical skin lesions, or a positive pathergy.
Recurrent oral ulcerations are categorized as minor aphthous, major aphthous, and
herpetiform ulcerations that recurred at least three times in a 12-month period.
Recurrent genital ulcerations are defined as aphthous ulceration and scarring. Eye lesions
were defined as anterior uveitis, posterior uveitis or cells in the vitreous on slit lamp
examination; and retinal vasculitis. Compatible skin lesions included erythema nodosum,
pseudofolliculitis, papulopustular lesions, and aceneiform nodules in post-adolescent patients
not receiving corticosteroids. A positive pathergy test of cutaneous hypersensitity was defined
as positive when a sterile pustule developed after twenty four to forty-eight hours at the site of
a needle prick to the skin [174]. Although the usual onset of BD is in the third or fourth
decade of life, pediatric-onset cases have been described [175, 176]. Uluduz and colleagues
[175] studied two large Istanbul BD cohorts totaling 728 patients, ascertaining and comparing
pediatric-onset (26 patients) and adult adult-onset (702 patients)-neurological (NBD) that
differed in male predominance and more frequent dural venous sinus thrombosis in pediatric
patients compared to parenchymal neurological involvement in adults. Citirik and colleagues
[176] described ocular findings in 34 pediatric patients that included panuveitis, posterior and
anterior uveitis respectively in 53%, 32% and 15% of patients. Other ocular findings included
cataracts in 59%, posterior synechiae in 24%, postoperative capsular opacification 24%,
vitreous condensation after vitritis in 50%, optic atrophy in 30%, cystoid macular edema
15%, narrowed or occluded retinal vessels after retinal phlebitis and branched retinal
occlusions in 6%, neovascularization of the disk and phthisis bulbi each in 3% of patients.
Arai and colleagues [177] described the postmortem findings in a young man with BD with
relapsing unilateral uveitis, sensorineural hearing loss, slight fever, and progressive CNS and
autonomic nervous system involvement that included multifocal brainstem and cerebellar
necrotic foci, perivascular neutrophilic inflammation, and perivasculitis.

Cortical venous sinus thrombosis (CVST) in BD most commonly presents with
symptoms and signs of increased intracranial pressure with a rarity of venous infarcts.
Prothrombosis when present, is presumed to commence as an endothelial disturbance.
The treatment of BD-related CVST includes consideration of anticoagulation and
corticosteroids alone or in association with another immunosuppressant agent.

Complimentary Contributor Copy