and tacrolimus are alternative agents for patients who are unresponsive to or intolerant of first-line agents. Chlorambucil is an effective option, but is now considered a second-line agent, and should be used with caution because of its potential for toxicity.

Recently, several case series have reported successful control of refractory ABD using anti-tumor necrosis factor (TNF) drugs infliximab and adalimumab.20,21 Intravitreal triamcinolone acetonide (IVTA) injections have been shown to resolve CME due to ABD.22 A novel localized treatment modality for ABD and other posterior uveitis disorders is the fluocinolone acetonide implant (Retisert, Bausch & Lomb, Rochester, NY), which has been demonstrated to reduce the frequency of uveitis recurrence, improve visual outcome, and reduce the need for adjunctive therapy in patients with noninfectious posterior uveitis.23 Potential adverse effects of IVTA and fluocinolone acetonide implant include cataract progression and glaucoma. The choice of which drug to use will depend on individual features such as tolerance and response to treatment.

BIRDSHOT RETINocHOROIDOPATHY

Diagnostic features

BSCR is characterized by bilateral, multiple, hypopigmented postequatorial retinal pigment epithelial (RPE) and choroidal inflammatory lesions associated with low-grade inflammation. The main clinical diagnostic criteria for BSRC include: (1) bilateral disease; (2) at least three peripapillary “birdshot lesions” inferior or nasal to the optic disc in one eye; (3) low-grade anterior-segment intraocular inflammation (<1+ cells); and (4) low-grade vitreous inflammatory reaction (<2+ vitreous haze). Supportive findings for BSCR include: presence of the HLA-A29 haplotype, retinal vasculitis, and CME. Exclusion criteria for BSRC include keratic precipitates, posterior synechiae, and presence of other inflammatory diseases that can cause multifocal choroidal lesions.24 BSCR patients may present with disturbances in night and color vision as well as decreased visual acuity.

ICGA usually demonstrates retinal and choroidal spots more numerous than clinically detected or seen on FA and may be more helpfulinassessingdiseaseactivityandprogression.25 Electroretinogram abnormalities were associated with recurrence of inflammation26 and improvement with therapy,27 and may be a useful adjunct in therapeutic decision making. Visual field (VF) abnormalities appear to be associated with retinal damage, and automated VF testing may be an objective measure to monitor activity.28 The most common VF patterns reported were multiple foci and arcuate defects. Optical coherence tomography (OCT) can be used to evaluate the macula in BSCR to determine causes

of central vision loss such as macular thinning,29 CME, epiretinal membrane, and choroidal neovascularization (CNV). BSCR must be differentiated from the white-dot syndromes and other causes of multifocal choroidal lesions (Table 22.3).

Treatment modalities

Periocular steroids are beneficial in acute exacerbation of inflammation and in the treatment of CME. However, the use of corticosteroid-spar- ing immunosuppressive agents is recommended for the treatment of BSCR because progressive deterioration of retinal function and poor visual prognosis have been associated with corticosteroid monotherapy. Low-dose azathioprine has been reported to be more effective than corticosteroids but does not lead to a durable remission. A combination of mycophenolate mofetil and CSA has been recommended as first-line therapy for BSCR.30 Alternative treatment agents for BSCR include intravenous immunoglobulin,31 and cytokine inhibition with infliximab32 and daclizumab.33

MULTIFOCAL CHOROIDITIS AND

PANUVEITIs (MCP)

MCP is a type of idiopathic posterior-segment chorioretinal inflammatory condition which exhibits: (1) bilateral, multiple (up to several hundred) choroidal lesions ranging in size from 50 to 350 m, arranged singly, in clumps or in linear clusters, situated in the posterior pole and/ or the periphery; (2) vitritis; (3) absence of systemic disease; (4) absence of features associated with other ocular diseases (e.g., HLA-A29 and abnormal electrocardiogram suggesting BSRC); (5) peripapillary changes, atrophy, or pigmentation; and (6) anterior uveitis. MCP predominantly affects young, healthy female adults and has no familial or racial predilection. Patients may complain of decreased visual acuity, photophobia, floaters, and photopsia usually affecting both eyes. MCP is a significant cause of visual impairment and visual loss: up to 38% of patients having 20/200 or worse visual acuity were reported in one series. The retinal complications of MCP include CNV, which is the most common cause of vision loss, as well as epiretinal membrane and CME.

FA will demonstrate the yellow or gray active lesions of MCP to be hypofluorescent in the early frames and hyperfluorescent in the late frames. CNV is a complication of MCP which can be confirmed by FA or ICGA. VF testing may show blind-spot enlargement or central scotoma. Electroretinography may demonstrate depressed rod and cone function.