Birdshot retinochoroiditis: long term follow-up of a chronically progressive disease

doi:10.1016/S0002-9394(02)01350-8

American Journal of Ophthalmology

Volume 133, Issue 5, May 2002, Pages 622-629

https://doi.org/10.1016/S0002-9394(02)01350-8 Get rights and content

Abstract

PURPOSE: To determine the long-term course of birdshot retinochoroiditis by reviewing patient records from The University of Iowa Hospitals and Clinics.

DESIGN: A descriptive case series.

METHODS: We conducted a retrospective review of 19 patients seen at The University of Iowa for birdshot retinochoroiditis. Inclusion criteria were set before review. Goldmann perimetry isopters were converted to an area measurement in steradians and the I2e and I4e isopters were evaluated at each time point. The visual acuity, electroretinography (ERG), and visual field findings were compared to the clinical appearance of the fundus.

RESULTS: Follow-up ranged from one visit to 220 months. Of the 14 patients who were tested, all were HLA-A29-positive. Seven patients were followed for ≥60 months. Eleven patients were followed for ≥30 months. The initial visual acuity was 20/50 or better in 36 of 38 eyes and 20/60 and 20/80 in the remaining two. Visual acuity was worse than 20/50 in three of 22 eyes followed for more than 30 months. Visual field data demonstrated progressive loss of area for either the I4e or I2e isopters in six of seven patients who were followed for ≥60 months. Multiple ERGs were performed over time on eight of 19 patients; seven of eight patients demonstrated progressive loss of electrophysiologic indices.

CONCLUSION: Retinal function in birdshot retinochoroiditis deteriorated progressively over a period of years despite stable visual acuity. Late in the course of disease, visual acuity may be lost due to chorioretinal atrophy in the posterior pole. Visual acuity alone is not an adequate parameter with which to monitor disease activity and may falsely suggest that a patient is stable or doing well. Intermittent treatment of the inflammatory exacerbations did not prevent progressive visual loss. Other treatment strategies such as prolonged corticosteroid or immunosuppressive treatment should be investigated for patients with birdshot retinochoroiditis.

Section snippets

Methods

The study design is a retrospective case review of 19 birdshot retinochoroiditis patients seen in a referral vitreoretinal clinic of the Department of Ophthalmology and Visual Sciences at The University of Iowa Hospitals and Clinics between 1982 and 1999. Patient cases were reviewed for the following inclusion criteria: (1) multifocal, oval yellow patches in the midperipheral retina without hyperpigmentation, often distributed in a radiating pattern outwards from the optic nerve head; (2)

Results

Nineteen patients with birdshot retinochoroiditis were identified (Table 1). Fourteen patients were HLA-A29-positive; five patients were not HLA-tested, but had the classic clinical picture. Mean age at presentation was 44.8 years (median, 43 years; range 30–76 years). There were 10 women and nine men. Mean follow-up time for patients with more than one visit was 82.5 months (median 66 months; range 2–269 months). Eleven patients had ≥ 30 months of follow-up and seven had ≥ 60 months

Discussion

In the initial series of Ryan and Maumenee, few patients demonstrated visual loss to 20/200 or worse.1 Much of the visual loss in other reports has been attributed to cystoid macular edema, serous detachment of the macula, or optic atrophy. Choroidal neovascularization has also been reported as a cause of decreased central vision.15 However, this complication is felt to be rare (6.5%)10 and only 5.3% in our series (two of 38 eyes) developed choroidal neovascularization during the course of

Acknowledgements

This work was supported in part by Research to Prevent Blindness, Inc., New York, NY.

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Treatment Outcomes in Birdshot Chorioretinitis: Corticosteroid Sparing, Corticosteroid Discontinuation, Remission, and Relapse
2022, Ophthalmology Retina
To describe treatment-related outcomes among patients with birdshot chorioretinitis (BSCR).
Retrospective cohort study.
Patients diagnosed with BSCR at 2 tertiary care academic medical centers.
Clinical and treatment-related data were collected for all patients with BSCR diagnosed between 2003 and 2017 at the 2 centers and for each eye at each clinical visit.
Four outcomes were considered: (1) corticosteroid-sparing success, defined as inactive disease and prednisone dose of ≤7.5 mg/day; (2) corticosteroid-discontinuation success, defined as inactive disease and discontinuation of prednisone; (3) sustained drug-free remission, defined as inactive disease off all medications for ≥3 months; and (4) relapse of BSCR after remission.
A total of 107 patients with BSCR were identified, of whom 94 had follow-up data. Corticosteroid-sparing success was achieved in 95.4% of the oral corticosteroid-treated patients at a rate of 0.60 successes per person-year (PY) (95% CI: 0.50/PY, 0.70/PY). The median time to corticosteroid-sparing success was 12 months. Corticosteroids were discontinued successfully in 76.5% of oral corticosteroid-treated patients (rate = 0.28/PY; 95% CI: 0.21/PY, 0.35/PY). The median time to successful corticosteroid discontinuation was 2.0 years. A sustained drug-free remission was achieved in 24 patients (rate = 0.06/PY; 95% CI: 0.04/PY, 0.09/PY), with approximately 25% of patients achieving remission by 4 years of follow-up. Relapse of inflammation in patients after achieving a sustained, drug-free remission occurred at a rate of 0.24/PY (95% CI: 0.14/PY, 0.37/PY).
Successful corticosteroid sparing and discontinuation was achieved in the majority of patients with BSCR. Remission occurred less often, but data were limited by the time needed to induce a remission (4 years) and the amount of follow-up (median, 4.6 years). The relapse rate after a remission was 0.24/PY.
Classification Criteria for Birdshot Chorioretinitis
2021, American Journal of Ophthalmology
To determine classification criteria for birdshot chorioretinitis.
Machine learning of cases with birdshot chorioretinitis and 8 other posterior uveitides.
Cases of posterior uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the infectious posterior uveitides / panuveitides. The resulting criteria were evaluated on the validation set.
One thousand sixty-eight cases of posterior uveitides, including 207 cases of birdshot chorioretinitis, were evaluated by machine learning. Key criteria for birdshot chorioretinitis included a multifocal choroiditis with (1) the characteristic appearance of a bilateral multifocal choroiditis with cream-colored or yellow-orange, oval or round choroidal spots (“birdshot” spots); (2) absent to mild anterior chamber inflammation; and (3) absent to moderate vitreous inflammation; or multifocal choroiditis with positive HLA-A29 testing and either classic “birdshot spots” or characteristic imaging on indocyanine green angiography. Overall accuracy for posterior uveitides was 93.9% in the training set and 98.0% (95% confidence interval 94.3, 99.3) in the validation set. The misclassification rates for birdshot chorioretinitis were 10% in the training set and 0% in the validation set.
The criteria for birdshot chorioretinitis had a low misclassification rate and seemed to perform sufficiently well for use in clinical and translational research.
Recommendations statement on the immunosuppressive treatment of non-infectious, non-neoplastic, non-anterior uveitis
2020, Medicina Clinica
Desarrollar recomendaciones basadas en la mejor evidencia disponible y experiencia sobre el uso de inmunomoduladores en pacientes con uveítis, las uveítis intermedias (UI), posteriores (UP) y panuveítis (PanU) no infecciosas, no neoplásicas.
Se seleccionó un grupo multidisciplinar de 5 expertos, que definió el alcance, usuarios y apartados del documento. Posteriormente, se realizó una revisión sistemática de la literatura sobre la eficacia y seguridad de los inmunomoduladores en pacientes con UI, UP y PanU no infecciosa, no neoplásica. Se generaron 34 recomendaciones en base a la evidencia encontrada en la revisión sistemática y a la experiencia de los expertos. Mediante la metodología Delphi, el grado de acuerdo con las recomendaciones se extendió a 25 expertos más que votaron según una escala de 1 (total desacuerdo) a 10 (total acuerdo). El acuerdo se definió como una puntuación ≥7 en al menos el 70% de los participantes. El nivel de evidencia y grado de recomendación se clasificaron según el modelo del Center for Evidence Based Medicine de Oxford.
Se aceptaron las 34 recomendaciones generadas tras 2 rondas Delphi (se modificaron 3 recomendaciones). Se incluyen recomendaciones específicas para pacientes con UI, UP y PanU no infecciosa, no neoplásica, así como para distintas líneas de tratamiento.
En los pacientes con UI, UP y PanU no infecciosas, no neoplásicas, estas recomendaciones pueden ayudar en la toma de decisiones terapéuticas, dada la ausencia de estudios con potencia estadística suficiente, u otros algoritmos universalmente aceptados sobre los que apoyar dichas decisiones.
To generate recommendations on the use of immunomodulators in patients with non-infectious, non-neoplastic intermediate uveitis (IU), posterior uveitis (PU) and panuveitis (PanU) based on best evidence and experience.
A multidisciplinary panel of 5 experts was established, who defined the scope, users, and sections of the document. A systematic literature review (SLR) was performed to assess the efficacy and safety of immunomodulatory drugs in patients with non-infectious, non-neoplastic, non-anterior uveitis. The results of the SLR were presented and discussed during an expert meeting in which 34 recommendations were generated. The level of agreement with the recommendations was also tested in 25 additional experts following a Delphi process. Recommendations were voted from 1 (total disagreement) to 10 (total agreement). We defined agreement if at least 70% of the experts voted ≥7. The level of evidence and grade or recommendation were assessed using the Oxford Centre for Evidence-based Medicine Levels of Evidence.
The SLR included 33 articles. The 34 recommendations were accepted after 2 Delphi rounds (3 of them were modified after the first round). They include specific recommendations on patients with non-infectious, non-neoplastic, PU and PanU, as well as different treatment guidelines.
In patients with non-infectious, non-neoplastic, non-anterior uveitis these recommendations might help treatment decision making, due to the lack of robust evidence or other globally accepted algorithms.
Five-Year Trends in Multifocal Electroretinogram for Patients With Birdshot Chorioretinopathy
2019, American Journal of Ophthalmology
The aim of this study is to investigate temporal trends in multifocal ERG (mfERG) parameters and analyze their relationships with anatomic and functional markers in patients with birdshot chorioretinopathy (BSCR).
Prospective observational case series.
Sixteen BSCR patients were include and underwent 2 standardized follow-up (FU) visits within 5 years following a baseline examination, including mfERG, visual acuity (VA), visual field (VF), Lanthony desaturated panel D-15 test for color vision, quality of life (QoL), fluorescein and indocyanine green angiography, and optical coherence tomography (OCT).
A significant trend toward a decrease in absolute N1 amplitude values was observed over the follow-up period (P < .001) while N1 implicit time remained unchanged. In contrast, P1 amplitude decreased (P < .001) and P1 implicit time increased (P < .001) over the same period. No significant temporal change was found for VA, color vision score, foveal threshold, mean deviation of VF, and QoL. After adjusting for time to FU, increasing N1 and P1 IT were both associated with decreasing values of logMAR, foveal threshold, and QoL and with increasing color vision score and mean deviation of VF. A significant relationship was observed between decreasing P1 amplitude values and increasing mean deviation of VF. Lower absolute values of N1 amplitude were associated with venous vasculitis, whereas lower P1 amplitude values correlated with alteration of the outer retina in OCT.
Progressive deterioration in mfERG during a 5-year period is detected in BSCR, whereas classical functional test results were unchanged. This study suggests a better sensitivity of mfERG in monitoring the retinal function of BSCR patients.
Immunosuppression for the Uveitides
2018, Ophthalmology
The uveitides are a collection of more than 30 diseases characterized by intraocular inflammation. Many cases of juvenile idiopathic arthritis–associated uveitis, many cases of intermediate uveitis, and most cases of posterior and panuveitides requiring treatment are treated with corticosteroids and immunosuppression. Disease-specific, time-updated modeling of clinical data for several uveitides suggests superior prevention of ocular complications and visual outcomes with immunosuppression. These studies also suggest that oral corticosteroids at doses low enough for safe long-term therapy (i.e., <7.5 mg/day) are ineffective, implying that immunosuppression should be part of the initial regimen. The Multicenter Uveitis Steroid Treatment (MUST) Trial and Follow-up Study was a randomized comparative effectiveness trial comparing systemic therapy with oral corticosteroids and immunosuppression with regional corticosteroid treatment. It demonstrated that, when used properly, oral corticosteroids and immunosuppression can be given safely for up to 7 years with no evident increased risk of systemic side effects compared with regional corticosteroid therapy, except for greater antibiotic use for infections. The Systemic Treatment for Eye Diseases (SITE) Cohort Study suggested long-term safety for this approach, when the immunosuppressive agents were antimetabolites or calcineurin inhibitors. Thus, oral corticosteroids and immunosuppression may be a preferred initial therapy for many noninfectious, intermediate, posterior, and panuveitides. Nonalkylating-agent immunosuppression has a low rate of sustained, drug-free remissions, <10%/year. Nonalkylating-agent immunosuppression for >3 years with control of the inflammation for >2 years is associated with a decreased risk of relapse after discontinuing immunosuppression. Alkylating agents can induce sustained drug-free remissions but likely increase the lifetime risk of cancer. Biologics, which target specific cytokines and pathways, hold promise for the future. Monoclonal antibodies directed against tumor necrosis factor (TNF)-α have been studied most often, and one, adalimumab, is U.S. Food and Drug Administration approved for the treatment of noninfectious, intermediate, posterior, and panuveitides.
Interferon-alpha2a and Systemic Corticosteroid in Monotherapy in Chronic Uveitis: Results of the Randomized Controlled BIRDFERON Study
2017, American Journal of Ophthalmology
Macular edema is the leading cause of vision loss in bilateral chronic noninfectious posterior uveitis, and is currently being treated using corticosteroids, immunosuppressive agents, and biotherapies. The aim of this trial was to assess and compare the efficacy and safety of corticosteroids and interferon-α (IFN-α) in adults with such conditions.
Randomized controlled trial.
Subjects: Adult patients with bilateral posterior autoimmune noninfectious and nontumoral uveitis complicated by macular edema in at least 1 eye. Intervention: Patients received either subcutaneous IFN-α2a, systemic corticosteroids, or no treatment for 4 months. The efficacy and safety were assessed for up to 4 months. Main Outcome Measures: The main endpoint was the change of the central foveal thickness (CFT) obtained by optical coherence tomography.
Forty-eight patients were included. In intention-to-treat analysis, the median CFT change showed no significant difference. However, the per-protocol analysis showed a significant difference between groups for both eyes (OD and OS), and for the worse and better eyes. Statistically significant difference was found between the control and corticosteroid groups for the OD (P = .0285), and between the control and IFN-α groups for the OD (P = .0424) and worse eye (P = .0354). Serious adverse events occurred in 2 patients in the IFN group, in 1 patient in the corticosteroid group, and in 2 patients in the control group and were completely resolved after switch.
IFN-α and systemic corticosteroids, compared with no treatment, were associated with significant anatomic and visual improvement shown in the per-protocol study.

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¹: Nancy J. Christmas and Kean T. Oh are Heed-Knapp Foundation Fellows for 1999–2001. Kean T. Oh is a Ronald G. Michel’s Foundation Fellow for 1999–2000.

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Birdshot retinochoroiditis: long term follow-up of a chronically progressive disease

Abstract

Section snippets

Methods

Results

Discussion

Acknowledgements

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Ophthalmology

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Vitiliginous chorioretinitis

Arch Ophthalmol