Page 379 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Ophthalmologic and Neuro-Ophthalmologic Aspects of Vasculitides 353

necrotizing scleritis and peripheral ulcerative keratitis associated with Hepatitis C virus
(HCV) infection.

Myers and colleagues [151] described in a 44-year-old man with chronic HCV-associated
CV who developed sudden loss of vision in his left eye and abdominal pain. Fundoscopy
revealed peripapillary cotton-wool spots and superficial retinal whitening in the macula. FFA
revealed retinal vascular non-perfusion in the left macular and peripapillary region, but not in
the periphery, consistent with Purtscher retinopathy. The authors proposed that the
retinopathy developed as a result of complement-mediated microembolism leading to
vaso-occlusion. Sauer and co-workers [152] documented a patient with Purtscher-like
retinopathy associated with HCV-associated CV with complaints of visual loss. Purtscher
retinopathy and hypertensive retinopathy were described by Gorevic and colleagues [153]
among 22 patients with CV, 14 (63.6%) of whom had hypertension, and 8 (36.4%) with
hypertensive retinopathy; the latter likely resulted from CV-mediated, renovascular
hypertension.

Central serous chorioretinopathy is another ocular complication of CV. Cohen and
colleagues [154] reported two patients with CV who presented with serous retinal and retinal
pigment epithelial detachments resembling central serous chorioretinopathy. The authors
postulated that the increased protein content of the choroid in patients with CV caused an
abnormal excess of interstitial fluid to accumulate in the subretinal pigment epithelium or
subretinal space resulting in retinal pigment epithelial detachment.

The treatment of CV consists of corticosteroids plus an alkylating agent which is
associated with induction of remission in up to 62% of patients. Rituximab plus
corticosteroids has more recently been found to be beneficial producing induction of
remission in up to 64% of patients. Though the combination of rituximab and corticosteroids
is the most effective treatment for CV, it is associated with infection in 42% [145] of patient
so treated. In those with HCV-associated CV, treatment of HCV with pegylated IFN and
ribavirin in addition to rituximab is the optimal treatment for most [155]. Nicholson and
Sobrin [149] described a patient with CV who presented with severe anterior uveitis who did
not respond to corticosteroids but improved with plasmapheresis and rituximab. Kedhar and
co-workers [150] found that necrotizing scleritis and peripheral ulcerative keratitis due to CV
improved with high-dose prednisone, pegylated interferon, ribavirin, and cyclophosphamide.
Myers and colleagues [151] described successful treatment of Purtscher retinopathy with
plasmapheresis, prednisone and cyclophosphamide leading to improvement in left eye VA
from 1/200 to 20/200, and resolution of retinal whitening, cotton-wool spots and retinal
hemorrhages observed on fundoscopy in six months.

Central serous chorioretinopathy, another ocular manifestation of CV, responded to
treatment with laser photocoagulation so noted by Cohen and colleagues [154] in two patients
who achieved near normal VA in both eyes.

IgA Vasculitis

This small vessel vasculitis is characterized by IgA IC deposition, in addition to
nonthrombocytopenic palpable purpura, abdominal pain and arthritis. It is one of the most
common vasculitides in children, with an annual incidence rate between 10-18 per 100,000
children per year [156, 157]. IgAV has a peak incidence in those under age 6, is slightly more

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