Page 380 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

354 Sarah Conway and David S. Younger

common in males, and more frequent during winter months [156-158]. The pathology of
IgAV is characterized by infiltration of small blood vessels with polymorphonuclear
leukocytes, and the presence of leukocytoclasia [156].

The ACR diagnostic criteria have found that the presence of two or more of the following
criteria were 89.4% diagnostically sensitive and 88.1% specific for IgAV including, age at
onset before 20 years, palpable purpura, acute abdominal pain, and biopsy showing
granulocytes around arterioles or venules [159].

Ocular manifestations of IgAV are rare, but case reports have documented a range of
ophthalmologic complications. Recurrent episcleritis was one of the first ocular complications
of IgAV in a 14-year-old girl who developed photophobia and intermittent ocular pain 5
weeks after the onset of joint symptoms [160], ophthalmologic evaluation of whom revealed
episcleritis and engorgement of the episcleral vessels.

The association between anterior uveitis and IgAV was first reported by Yamabe and
colleagues [161] in the description of a patient with nephritis, anterior uveitis and keratitis
later. Muqit and co-workers [162] described a 42-year-old man with HSP complicated by
keratitis and granulomatous anterior uveitis. Erer and co-workers [163] reported a 39-year-old
man with IgAV who presented with three episodes of anterior uveitis, one of which was
bilateral, and unilateral episodes of each eye. Kaur and colleagues [164] documented bilateral
anterior uveitis associated with IgAV in an 11-year-old boy with a 4 year history of eye pain
and photosensitivity. Uveitis in IgAV is thought to be due to circulating IC, which reach the
eye and deposit in uveal tissues. IC can deposit in vascular endothelial cells, pigmented
epithelial cells, and corneal endothelial cells, expressing adhesion molecules that allows
leukocytes to migrate to the uveal tissue and cornea causing injury [165].

Although IgAV is not typically associated with frank bleeding, bilateral subperiosteal
orbital hematomas are a recognized complication so noted by Ma?luf and colleagues [166]
in a 5- year-old-boy who presented with bilateral exophthalmos and bilateral upper eyelid
ecchymosis.

Wu and colleagues [167] reported a 6-year-old girl who developed sudden vision loss,
and later found on fundoscopy to have a cherry red spot with severe retinal edema of the
macula and peripalliary area of the eyes, with disc edema and venous engorgement consistent
with bilateral central retinal artery occlusion. Central retinal vein occlusion was reported in a
12-year-old boy with IgAV [168].

Other rare ocular manifestations include AION [169] and acute visual loss due to
bilateral cystoid macular edema and cotton wool spots [170].

The treatment of IgAV varies with disease severity with milder cases responsive to
supportive therapy and self-resolve in 6 to 16 weeks, and more severe involvement requiring
systemic corticosteroids and IVIg [171]. Ocular manifestations of IgAV resolve with a
combination of systemic and topical corticosteroids. A patient with episcleritis due to IgAV
responded to corticosteroids, but had exacerbations of episcleritis whenever the alternate day
prednisone dose was tapered to less than 30 mg (160). Erer and co-workers [163] described a
patient with anterior uveitis who had three recurrent episodes of anterior uveitis, each which
resolved within days of treatment with topical corticosteroids. Kaur and colleagues [164]
reported on an 11-year-old male with anterior uveitis that responded to systemic
prednisolone, topical steroids and homatropine eye drops.

AION-associated IgAV responds moderately well to corticosteroid treatment as noted by
Chuah and Meaney (169) in the care of a 54-year-old man with AION whose VA improved

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