Birdshot Chorioretinopathy Research Articles

Clinical features of intraocular inflammation in Hokkaido, Japan

We aimed to investigate the clinical features of intraocular inflammation/uveitis in Hokkaido, Japan. We retrospectively reviewed the medical records of 1240 uveitis patients (511 men, 729 women) who visited Hokkaido University Hospital, Sapporo, Japan between 1994 and 2003. Mean age at disease onset was 41.7 +/- 17.8 years in men and 45.7 +/- 18.3 years in women. Anterior, posterior and combined anterior and posterior segment intraocular inflammation accounted for 45.1%, 4.7% and 50.2% of cases, respectively. Sarcoidosis was the most frequent aetiology (14.9%), followed by Vogt-Koyanagi-Harada (VKH) disease (9.7%) and Behçet's disease (6.7%). Aetiologies in 49.8% patients were unknown. In sarcoidosis, women represented 72.4% of patients, and disease onset occurred at 35.1 +/- 19.0 years of age in men and 50.3 +/- 16.5 years in women. In VKH disease, 54.2% of patients were women, and disease onset took place at 45.9 +/- 15.8 years in men and 46.4 +/- 14.1 years in women. In Behçet's disease, men accounted for 56.6% of patients, and disease onset occurred at 35.5 +/- 8.5 years in men and 44.5 +/- 11.5 years in women. Women were more prone to developing sarcoidosis compared with men. By contrast, men were more prone to developing Behçet's disease. The mean age at disease onset in both sarcoidosis and Behçet's disease was significantly lower in men than in women.

Clinical Features, Treatment Methods, and Evolution of Birdshot Chorioretinopathy in 5 Different Families

To report familial cases of birdshot chorioretinopathy (BSCR). Retrospective, observational case series. Five families with 2 members in each (10 patients) with BSCR were included in this case series from a cohort of 225 patients followed up for BSCR. A review of medical and angiographic records of familial cases of BSCR was performed. We described and compared in each family the histocompatibility leukocyte antigen (HLA) typing, age at onset, ethnic group, clinical features, fluorescein and indocyanine green angiography results, evolution of disease, and treatment used with efficiency. HLA-A29 and HLA-B44 results were positive in 100% of patients tested. Mean age at onset was 46.5 years. All patients were White. Corticosteroids were required in 7 patients, and 3 patients had no treatment because of mild or moderate lesions. Cyclosporine A was administered in 2 patients from the same family. Period of follow-up ranged from 1 to 32 years (median, 7 years). Familial forms of BSCR remain rare and do not justify systematic examination in relatives of patients with BSCR.

The Spectrum of Fundus Autofluorescence Findings in Birdshot Chorioretinopathy

Objective. To describe the diverse patterns observed with the use of autofluorescence fundus photography (FAF) in patients with Birdshot chorioretinopathy (BSCR). Methods. A chart review of patients with BSCR seen at the Massachusetts Eye Research and Surgery Institution, who had autofluorescence fundus photography. The data obtained included age, gender, presence of the HLA-A29 haplotype, and current treatment. Results. Eighteen eyes with HLA-A29 associated BSCR were included. Four eyes presented with active inflammation. Correspondence of the lesions noted in the colour fundus photograph was observed in 3 eyes which were more easily identified with the FAF. Fifteen eyes had fundus lesions more numerous and evident in the FAF than in the colour fundus photograph. Conclusion. Because FAF testing provides valuable insight into the metabolic state of the PR/RPE-complex, it may serve as a useful noninvasive assessment tool in patients with posterior uveitis in which the outer retina-RPE-choriocapillaries-complex is involved.

Bilateral choroidal neovascularization in birdshot retinochoroidopathy treated with intravitreal injections of triamcinolone and bevacizumab.

To describe a patient with birdshot retinochoroidopathy (BRC) with bilateral choroidal neovascularization (CNV) who was treated with intravitreal injection of bevacizumab and antiinflammatory medications. Interventional case report. A 35-year-old woman with bilateral CNV associated with BRC. The patient was treated with intravitreal injection of triamcinolone, photodynamic therapy, and intravitreal injection of bevacizumab in one eye, while the fellow eye was treated with intravitreal injection of triamcinolone. Immunosuppressive therapy was performed in the course of the treatment. Not only did the neovascularization respond, but the birdshot lesions vanished as well. BRC can have secondary CNV that, as in the current case, responds favorably to treatment. We serendipitously observed regression of the choroidal inflammatory lesions with intravitreal injection of triamcinolone.

Immune Response Genes in Uveitis

Uveitis is defined as an intraocular inflammation induced by different etiologies. Though the precise pathogenesis is still unknown, accumulating evidence shows that both innate and adaptive immune responses may be predominant mechanisms involved in the development of uveitis. Toll-like receptors have been shown to be expressed in the human eye and play an important role in infectious uveitis. The NOD proteins, expressed mainly in the cytosol by APCs, recognize the products of bacteria and participate in the development of uveitis. HLA genes have been associated with some uveitis entities, including acute anterior uveitis (HLA-B27), Behcet disease (HLA-B51), birdshot retinochoroidopathy (HLA-A29), Vogt-Koyanagi-Harada syndrome (HLA-DR4), sarcoidosis, sympathetic ophthalmia, juvenile idiopathic arthritis, tubulointerstitial nephritis and uveitis (TINU) syndrome, and pars planitis (HLA-DR15). The exact mechanism whereby certain HLA genes predispose to a certain uveitis entity has not yet been elucidated. In addition, several studies have demonstrate that polymorphisms in certain immune response genes, such as tumor necrosis factor (TNF), MHC class I polypeptide-related sequence A (MICA), interleukin-1 (IL-1), and some chemokines, may contribute to the development of human uveitis. Polymorphisms in the gene coding for the costimulatory molecule known as cytotoxic T-lymphocyte antigen 4 (CTLA-4) were recently found in Chinese patients with VKH syndrome but not in patients with Behcet disease. Further developments in the unraveling of immune response genes may lead to a better understanding of human uveitis and will hopefully allow the development of novel treatment regimes.

Immunosuppressive therapy for ocular diseases

To identify advances in immunosuppressive therapy of ocular diseases since 2007. The biologics in current use include antitumour necrosis factor-alpha agents (infliximab, etanercept and adalimumab), cytokine receptor antibodies (daclizumab) and interferon-alpha2a. They are effective and comparatively well tolerated options in the treatment of refractory uveitis in both adults and children in the short term, except for etanercept. Daclizumab had a favourable outcome in treating birdshot chorioretinopathy but not in Behcet's disease. The uncertainty of their long-term results, their high costs as well as the necessity for repeated intravenous infusions in the case of infliximab limit their widespread use. Mycophenolate mofetil is another efficacious, fairly well tolerated and less costly immunosuppressant. It has the additional advantage of an oral formulation. T cell inhibitors, cyclosporine and tacrolimus, were found to be useful steroid-sparing drugs in allergic eye disease and dry eyes. A number of studies on less invasive sustained ocular drug delivery systems, including episcleral implants, nanospheres, and cyclodextrin particles, were conducted on animals with encouraging results. The armamentarium of immunosuppressive agents is constantly expanding and augurs well for the safe and effective treatment of ocular inflammation.

Contrast Sensitivity Among Patients with Birdshot Chorioretinopathy

To assess contrast sensitivity in patients with birdshot chorioretinopathy; to identify relationships between contrast sensitivity, other measures of visual function, clinical findings, and quality of life. Single-center, cross-sectional study. We measured contrast sensitivity in 63 patients (126 eyes) at four spatial frequencies (3, 6, 12, 18 cycles/degree [cpd]) using the CSV-1000E instrument (VectorVision, Greenville, Ohio, USA). Abnormal contrast sensitivity was defined as two standard deviations below the mean for population norms. Results at spatial frequency 12 cpd were compared to the following parameters in per-eye analyses: best-corrected visual acuity (BCVA); presence of eight specified symptoms; color vision; visual field parameters (foveal threshold, mean deviation); and optical coherence tomography parameters (central macular thickness, loss of the third highly reflective band). Results were compared to the National Eye Institute Visual Function Questionnaire (VFQ)-25 in per-patient analyses. Results were adjusted for age, disease duration, treatment, BCVA, and lens status. Contrast sensitivity (spatial frequency 12 cpd) was abnormal in 99 eyes (92%), and was related to poor BCVA (P = .0004) and the symptom of poor contrast sensitivity (P = .025). Among 38 eyes with normal BCVA (> or =1.0), 31 eyes (82%) had abnormal contrast sensitivity. There was a positive correlation between contrast sensitivity in better eyes and the VFQ-25 composite scores (r = 0.51; P < .001). Decreased contrast sensitivity is common in patients with birdshot chorioretinopathy and may occur in the absence of other visual changes. Contrast sensitivity may be a useful measure for clinical studies of birdshot chorioretinopathy and for monitoring patients with the disease.

Longitudinal Cohort Study of Patients with Birdshot Chorioretinopathy. V. Quality of Life at Baseline

To describe results of a vision-specific quality-of-life (QOL) questionnaire at baseline examination of 80 subjects in a longitudinal cohort study of birdshot chorioretinopathy and to identify relationships between these results and measures of visual function. Single-center, cross-sectional study. The National Eye Institute Visual Function Questionnaire-25 (NEI VFQ-25) was administered to all subjects. NEI VFQ-25 composite scores were compared with best-corrected visual acuity (BCVA), symptoms, color confusion scores, and parameters from automated perimetry (mean deviation [MD], total deviation) for the better eye (with regard to the factor being studied) of each subject. Selected measures of visual function were compared with the 12 subscale scores. The median composite score was 76.8 (range, 7.8 to 99.4). Worse composite scores were related to decreased BCVA (P = .030), but the correlation was weak, and subjects with normal BCVA (> or = 1.0) in both eyes had composite scores as low as 37.7. Lower composite scores were related to symptoms of blurry vision (P = .0097), nyctalopia (< .0001), poor contrast (P = .002), vibrating vision (P = .014), and poor peripheral vision (P = .007). Lower composite scores were related to worse MD (P = .005). Although nyctalopia and MD are related, each was associated with composites scores on multivariate analysis. Nyctalopia was associated with the largest number of subscales having decreased scores. Birdshot chorioretinopathy has an impact on vision-specific QOL. BCVA alone does not explain decreased vision-specific QOL in our cohort; changes in automated perimetry and symptoms seem to be important contributors to alterations in QOL that are independent of BCVA changes.

False negative antibody-based HLA-A29 typing in two patients with birdshot chorioretinopathy

A strong association between birdshot chorioretinopathy and HLA-A29 expression has been well established.1 In fact, some have suggested that HLA-A29 expression is essential to make the diagnosis of birdshot chorioretinopathy....

High-dose humanized anti-IL-2 receptor alpha antibody (daclizumab) for the treatment of active, non-infectious uveitis

Purpose This study was designed to provide preliminary data regarding the safety and efficacy of high-dose humanized anti-IL-2 receptor (daclizumab) therapy for the treatment of active intermediate, posterior or panuveitis. Methods Five patients were recruited into this non-randomized, prospective pilot study of high-dose intravenous induction daclizumab therapy given at doses of 8 mg/kg at day 0 and 4 mg/kg at day 14. Patients who did not meet a safety endpoint at the 3-week follow-up evaluation were given the option of continuing therapy with subcutaneous daclizumab at 2 mg/kg every 4 weeks for 52 weeks. The primary outcome assessed was a two-step decrease in vitreous haze at day 21. Secondary outcomes evaluated included best-corrected visual acuity, retinal thickness as measured by optical coherence tomography, retinal vascular leakage assessed by fluorescein angiography, anterior chamber and vitreous cellular inflammation. Results Four male patients and one female patient were enrolled. Diagnoses included birdshot retinochoroidopathy (two patients), Vogt–Koyanagi–Harada's disease, bilateral idiopathic panuveitis and bilateral idiopathic intermediate uveitis. By the 4th week, four of five patients demonstrated a two-step decrease in vitreous haze. The other participant did not meet this criterion until week 20, but all five patients maintained stability in vitreous haze grade throughout their follow-up periods. At enrollment, mean visual acuity (10 eyes in 5 patients) was 69.2 ETDRS letters and following treatment was 78.2 letters ( p < 0.12). Anterior chamber cell, vitreous cell, and vitreous haze also improved in the majority of eyes. Adverse events were generally mild except for one episode of left-lower lobe pneumonia requiring hospitalization and treatment. Conclusion This is the first demonstration that high-dose daclizumab can reduce inflammation in active uveitis. Daclizumab was well tolerated but there may be a potential increased risk of infection associated with immunosuppression. All five patients demonstrated a decrease in vitreous haze and measures of intraocular inflammation at final follow-up. The results of this small, non-randomized pilot study support the consideration of high-dose daclizumab therapy in cases of active posterior uveitis.

Anti-TNF-α Therapy in Patients with Chronic Non-Infectious Uveitis: The Experience of Jules Gonin Eye Hospital

The purpose of this study is to describe the experience of Jules Gonin Eye Hospital on the long-term outcome of anti-TNF-alpha therapy in chronic non-infectious uveitis. We identified and followed those patients with chronic non-infectious uveitis who received systemic anti-TNF-alpha therapy. Anti-TNF-alpha therapy was administered when no response had been obtained with classical immunosuppressive therapies or in the presence of severe rheumatoid disease. Fifteen patients (28 eyes), 7 male and 8 female (mean age, 43 years; range: 7 to 70 years) were identified. Diagnoses included HLA-B27-associated anterior uveitis (n = 4), sarcoidosis (n = 2), juvenile idiopathic arthritis (n = 2), idiopathic retinal vasculitis with uveitis (n = 2), pars planitis (n = 2), Adamantiades-Behçet disease (n = 1), birdshot retinochoroidopathy (n = 1), and Crohn's disease (n = 1). Mean duration of ocular disease was 8 years (range: 1 to 29 years). Treatment with infliximab (n = 11), etanercept (n = 2), or adalimumab (n = 2) was initiated. One patient with etanercept was switched to infliximab due to lack of clinical response. Clinical and angiographic regression of uveitis was observed within the first two months of therapy in all patients, and was maintained throughout the entire follow-up period (mean 18 months; range: 3 - 72 months). Recurrence was observed in 3 patients, and resolved after adjustment of therapy. Adverse events were recorded in only one patient (arterial hypotension). In this series of patients with chronic non-infectious uveitis, anti-TNF-alpha therapy was effective and safe. Further clinical studies are needed to determine an adequate duration of therapy.

Fundus Autofluorescence in Birdshot Chorioretinopathy

To investigate the characteristics of fundus autofluorescence in birdshot chorioretinopathy (BSCR). Retrospective, observational case series. Sixteen eyes of 8 consecutive patients with BSCR (3 men, 5 women). Color and autofluorescence photography and optical coherence tomograms of patients with BSCR seen in a referral practice were evaluated. Autofluorescent characteristics in BSCR. The 8 patients ranged in age from 35 to 73 years (mean, 56.9 years). Of the 16 eyes, 11 eyes (69%) of 6 patients had retinal pigment epithelium (RPE) atrophy as evidenced by hypoautofluorescent regions. Some of the hypoautofluorescent areas corresponded to the hypopigmented birdshot lesions, but the others did not necessarily show a correspondence. Eight eyes (50%) of 4 patients showed linear hypoautofluorescent streaks along the retinal vessels, most of which corresponded to visible changes at the level of the RPE. Placoid hypoautofluorescence in the macula was seen in 6 eyes (38%) of 3 patients and was correlated significantly with best-corrected visual acuity of 20/50 or less (P<0.05). Autofluorescence photography demonstrated the RPE atrophy, which was hard to see by other means of investigation. The areas of RPE atrophy did not necessarily correspond to the hypopigmented lesions, which suggested that both the choroid and the RPE can be affected independently. Retinal pigment epithelium atrophy in the macula may be an important cause of poor central visual acuity in eyes with BSCR.

Combination of KIR and HLA gene variants augments the risk of developing birdshot chorioretinopathy in HLA-A*29-positive individuals

Birdshot chorioretinopathy (BCR), a chronic ocular inflammatory disease with characteristic choroidal lymphocytic infiltrates, has been strongly associated with human leukocyte antigen (HLA)-A29. Although HLA-A29 occurs frequently in all populations, BCR affects only a small percentage of HLA-A29-positive Caucasians, indicating additional susceptibility factors for BCR. Discovery of HLA class I-specific killer cell immunoglobulin-like receptors (KIR) led to a series of epidemiological studies implicating KIR-HLA gene combinations in disease. Here, we characterized KIR-HLA pairs in BCR patients and controls carrying HLA-A*29 as well as controls lacking HLA-A*29. KIR-HLA pairs implicated for weak inhibition (KIR2DL2/3+HLA-C1 and KIR3DL1+HLA-Bw4(T80)) in combination with activating KIR genes associated with autoimmunity (KIR2DS2, 2DS3 and 2DS4) augment the risk of developing BCR in HLA-A*29-positive individuals. The reciprocal association of strong inhibitory pairs (KIR3DL1+HLA-Bw4(I80) and KIR2DL1+HLA-C2) in combination with those implicated in protection from infection (KIR3DS1+HLA-Bw4(I80) and KIR2DS1+HLA-C2) was observed in HLA-A*29-negative controls. These results suggest that a profound effect of KIR2DS2/S3/S4 in the absence of strong inhibition may enhance the activation of natural killer cells and T-cell subsets against intraocular self-antigens, thereby contributing to pathogenesis of BCR.

Combination of KIR‐HLA genotypes implicated in weak inhibition and autoimmunity augments risk for Birdshot Chorioretinopathy in HLA‐A29 positive individuals

HLA‐A29 is strongly associated with birdshot chorioretinopathy (BCR), a chronic ocular inflammatory disease with characteristic choroidal lymphocytic infiltrates. Although HLA‐A29 occurs frequently in all populations, BCR affects only a small percentage of HLA‐A29 positive Caucasians, indicating additional susceptibility factors for BCR. Discovery of HLA class I‐specific killer cell immunoglobulin‐like receptors (KIR) led to a series of epidemiological studies implicating KIR‐HLA gene combinations in disease. Here, we characterized KIR‐HLA pairs in BCR patients and controls carrying HLA‐A29 as well as controls lacking HLA‐A29. We found that combination of KIR‐HLA genotypes implicated for weak inhibition (KIR2DL2/3+HLA‐C1 and KIR3DL1+HLA‐Bw4T80) and autoimmunity (KIR2DS2/S3/S4) augments the risk of developing BCR in HLA‐A29 positive individuals. The reciprocal association of strong inhibitory pairs (KIR3DL1+HLA‐Bw4I80 and KIR2DL1+HLA‐C2) in combination with those implicated in protection from infection (KIR3DS1+HLA‐Bw4I80 and KIR2DS1+HLA‐C2) was observed in HLA‐A29‐negative controls.

Daclizumab for Treatment of Birdshot Chorioretinopathy

To report the outcomes for daclizumab in the treatment of birdshot chorioretinopathy (BSCR) refractory to traditional immunomodulatory therapy (IMT). We retrospectively reviewed medical records of 8 patients with BSCR whose disease was refractory to or who were intolerant of traditional IMT. All patients received 1 mg/kg of daclizumab intravenously at 2-week intervals initially at 1 referral uveitis practice. Main outcome measures were changes in visual acuity, vitreous inflammation, fluorescein angiographic pathologic features, electroretinography (ERG) parameters, concomitant IMT requirements, and adverse events. Over a mean follow-up of 25.6 months, 7 patients had either stabilization or improvement in visual acuity of both eyes and complete resolution of vitreous inflammation. Six patients had resolution of vasculitis on fluorescein angiography. The ERG 30-Hz implicit times and the bright scotopic amplitudes worsened in some patients despite abolition of clinically evident inflammation. Four patients were able to discontinue all other IMT and remain inflammation free while receiving only daclizumab treatment. Two patients developed adverse effects that led to daclizumab treatment discontinuation. Daclizumab therapy was effective in stabilizing vision and decreasing inflammation in most patients with BSCR. The ERG parameters continued to decline in some patients despite adequate inflammatory control. Regular serologic monitoring is critical to detect adverse events.

Demographic and Clinical Features of Uveitis in Tertiary Centers in Turkey

Purpose: To analyze the referral patterns and clinical features of uveitis in tertiary eye care centers in Turkey. Methods: Uveitis services of eight university clinics collected data on uveitis patients who presented during 2004. Data were collected on a form including a questionnaire on socio-demographic and clinical features which were documented at presentation. Results: A total of 761 patients (1187 eyes) with a mean age of 35.5 years were included in the study. Male to female ratio was 1.04:1. The majority of patients (89.6%) were from an urban population, 53% were referrals and 43% had had uveitis for more than one year. The most common type of uveitis was anterior uveitis (52.5%) followed by panuveitis (28.1%), posterior uveitis (12.7%) and intermediate uveitis (6.7%). In 56.8% of patients an etiological classification was established. The most common etiology was Behçet's disease (32.1%), followed by Fuchs' heterochromic iridocyclitis (5.1%) and ocular toxoplasmosis (4.7%). Among 48 pediatric patients uveitis associated with juvenile idiopathic arthritis and ocular toxoplasmosis both having equal frequencies (12.5%) were the most common diagnosis. These were followed by Behçet's disease with juvenile-onset (10.4%). At the time of presentation, 6.25% of the patients were legally blind (having visual acuity less than 0.1 in the better seeing eye). Conclusion: Compared to the uveitis series reported from other countries, Behçet uveitis was the leading cause of uveitis in this series. Uveitis associated with juvenile idiopathic arthritis and ocular toxoplasmosis were the most common cause of uveitis in childhood. On the other hand, entities like acquired immunodeficiency syndrome-related uveitis, presumed ocular histoplasmosis and Birdshot retinochoroidopathy were rare.

White-Dot-Syndrom

The white dot syndromes comprise a group of diseases with a suspected immunological background, which show no systemic manifestations. The characteristic inflammatory changes of the choroid and the retinal pigment epithelium are typically yellow-white foci beneath the retina. Diseases belonging to the white dot syndromes which will be discussed in this article are acute multifocal placoid pigment epitheliopathy (AMPPE), multiple evanescent white dot syndrome (MEWDS), birdshot retinochoroidopathy (BSRC), multifocal choroiditis with panuveitis (MFC/MCP), punctuate inner choroidopathy (PIC), acute zonal occult outer retinopathy (AZOOR) and serpiginous choroiditis, Neither the trigger mechanism nor the pathogenetic development is known with certainty for any of these diseases. Immunological reactions to previous viral infections coupled with a genetic predisposition seem to be a common denominator. Transitions between the individual diseases have also been described.

Genetics of Birdshot Chorioretinopathy

Birdshot chorioretinopathy is a bilateral, posterior uveitis that affects primarily Caucasians. Although the genetic basis of disease is not completely understood, certain factors are known, such as the strong association with human leukocyte antigen (HLA)-A29. However, given the prevalence of the HLA-A29 allele in the general population, if it were the only factor required for disease, many more people would have birdshot chorioretinopathy. It has been suggested that some environmental factor may trigger this disease entity in those who are genetically predisposed based on inheritance of the HLA-A29 allele.

Atypical Bartonella hensalae Chorioretinitis in an Immunocompromised Patient

Purpose: To report an atypical case of chorioretinopathy in a patient with bilateral renal transplantations. Methods: A 55-year-old female was referred for management of birdshot chorioretinopathy. Ophthalmologic examination revealed bilateral yellowish, chorioretinal lesions with adjacent hemorrhages. Results: Angiography demonstrated lesions with hyperfluorescence, leakage, and diffuse macular edema. OCT showed intraretinal edema. Laboratory evaluation revealed IgG antibodies for Bartonella hensalae. Treatment with oral ciprofloxacin led to regression of lesions, resolution of macular edema, and improvement in visual acuity. Conclusion: Multifocal chorioretinal lesions associated with B. hensalae can be atypical ophthalmic manifestations of cat-scratch disease (CSD), which may occur in immunosuppressed patients. Recognition of underlying disease and appropriate therapy can lead to improved outcomes.

Sarcoid choroiditis simulating birdshot chorioretinopathy.

To report an atypical presentation of sarcoidosis simulating birdshot chorioretinopathy. Observational case report. Review of the clinical, laboratory, photographic, and histopathologic records of a patient with sarcoidosis. A 56-year-old white woman was evaluated for uveitis in both eyes. Dilated funduscopic examination of both eyes revealed numerous oval and irregular hypopigmented chorioretinal lesions located inferior nasally radiating from the optic nerve that were virtually identical to the typical funduscopic appearance of birdshot chorioretinopathy. The patient was lost to follow-up and returned 5 years later. In the interim, the patient underwent mediastinal lymph node biopsy that showed noncaseating granulomas consistent with sarcoidosis. The serum angiotensin-converting enzyme level was normal, and results of HLA- A29 testing were negative. Sarcoidosis can cause choroidal lesions similar to those of birdshot choroidopathy. Clinicians should consider sarcoidosis in the differential diagnosis of birdshot chorioretinopathy, and patients initially diagnosed with birdshot choroidopathy should also undergo workup for sarcoidosis.