Birdshot chorioretinopathy: Importance of early diagnosis and therapeutic individualization

Elisabet Martin Garcia; Leticia Rodríguez Vicente; José Javier Chávarri García; José Luis del Rio Mayor

doi:10.4103/PAJO.PAJO_25_19

CASE REPORT

Year : 2020 | Volume : 2 | Issue : 1 | Page : 3

Birdshot chorioretinopathy: Importance of early diagnosis and therapeutic individualization

Elisabet Martin Garcia, Leticia Rodríguez Vicente, José Javier Chávarri García, José Luis del Rio Mayor
Department of Ophthalmology, San Pedro Hospital of Logroño, Logroño, Spain

Date of Submission	28-Nov-2019
Date of Decision	05-Dec-2019
Date of Acceptance	05-Dec-2019
Date of Web Publication	23-Jan-2020

Correspondence Address:
Dr. Elisabet Martin Garcia
Department of Ophthalmology, San Pedro Hospital of Logroño, Logroño
Spain

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/PAJO.PAJO_25_19

Abstract

Introduction: Birdshot chorioretinopathy (BCR) is a bilateral and recurrent posterior uveitis with an uncertain natural history and visual prognosis. Currently, there is no consensus on the best treatment option and its total duration.
Clinical Cases: We present a series of three clinical cases of patients with BCR diagnosed in different stages of the disease, which demonstrates the clinical spectrum of this pathology and their treatment with different immunomodulatory drugs.
Conclusion: As shown in our series of cases, the early use of immunomodulatory drugs is recommended with a close monitoring and individualized treatment to achieve the best anatomical and functional results.

Keywords: Birdshot chorioretinopathy, posterior uveitis, inmunomodulatory drugs

How to cite this article:
Garcia EM, Vicente LR, Chávarri García JJ, del Rio Mayor JL. Birdshot chorioretinopathy: Importance of early diagnosis and therapeutic individualization. Pan Am J Ophthalmol 2020;2:3

How to cite this URL:
Garcia EM, Vicente LR, Chávarri García JJ, del Rio Mayor JL. Birdshot chorioretinopathy: Importance of early diagnosis and therapeutic individualization. Pan Am J Ophthalmol [serial online] 2020 [cited 2023 Mar 28];2:3. Available from: https://www.thepajo.org/text.asp?2020/2/1/3/276598

Introduction

Birdshot chorioretinopathy (BCR) is a bilateral and recurrent posterior uveitis.^[1] It is a pathology that is diagnosed almost exclusively among the Caucasian adult population with an average age of onset of 53 years and a slight predominance among women.^[2]

Being an autoimmune etiology, BCR is not related to any general disease but has a strong association with the histocompatibility antigen (HLA A29), although it has been observed that its isolated detection does not completely justify the susceptibility to the disease. Similarly, there are approximately 5% of BCA cases that are HLA A29 negative.^[3]

Patients report a bilateral and often asymmetric clinical presentation. Symptoms include blurred vision, myodesopsia, nyctalopia, and impaired color perception.^[4]

In the ophthalmological examination, the most characteristic finding is the appearance of oval and hypopigmented chorioretinal lesions of creamy appearance specially located in the nasal and juxtapapillary area. These lesions may not be clearly identified in the initial stages which makes early diagnosis difficult. Among the associated complications, cases of macular edema (63%), retinal vasculitis (40%), papillary edema (38%), epiretinal membrane (10%), choroidal neovascularization (6%), and optic nerve atrophy have been described (4%).^[5]

Clinical Cases

Case 1

A 34-year-old woman presented to an ophthalmologist with floaters and decreased visual acuity (VA) of 3 months of evolution.

On ophthalmologic examination, her best-corrected VA (BCVA) was 0.6 in the right eye (RE) and 0.8 in the left eye (LE). Biomicroscopically, she presented a mild Tyndall in the anterior chamber. On funduscopic examination of both eyes (BE), we found vitritis ++, slight papillary edema, and multiple chorioretinal cream lesions of <1 diameter of papilla in the posterior pole, more numerous in the nasal retina [Figure 1]. Macular optical coherence tomography (OCT) was normal. Signs of peripheral vasculitis were confirmed by fluorescein angiography (FA).

Figure 1: Multiple chorioretinal cream lesions in nasal retina in the right eye

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Given these findings, numerous complementary studies were requested. We observed an human HLA A29 positivity, and electro-oculogram (EOG) and electroretinogram (ERG) showed a slight decrease in response in photopic and scotopic conditions in the RE. The LE was normal.

The diagnosis of BCR was established by initiating treatment with 60 mg/day of oral prednisone. When the dose dropped to 30 mg/day, the patient accused a significant decrease in VA, and her BCVA was 0.5 in BE. Macular OCT showed macular edema in BE, being higher in the RE [Figure 2], the reason for which we decided to add to the treatment oral cyclosporin A at doses of 100 mg/12 h and an intravitreal dexamethasone implant (Ozurdex ^®) in BE.

Figure 2: Macular optical coherence tomography. Macular edema in the temporal zone

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One year after achieving stability, a taper off cyclosporin A was started until its complete suspension. After more than 2 years, the patient is stable [Figure 3].

Figure 3: Macular optical coherence tomography. Absence of macular edema after treatment

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Case 2

A 22-year-old male presented to the clinic with symptoms of floaters and dyschromatopsia in BE of a year of evolution.

On ophthalmologic examination, the BCVA was 0.8 in his RE and 0.9 in his LE. Anterior segment was normal. Fundus examination revealed vitritis ++, hyperemic papillae, creamy chorioretinal foci limited to the posterior pole of AO, and signs of peripheral vasculitis. Macular OCT was normal. FA revealed vascular leakage in early stages. In addition, chorioretinal lesions showed hypofluorescent in early stages and hyperfluorescent in late stages [Figure 4]a and [Figure 4]b. HLA A29 was positive. The EOG and ERG showed a decrease in response in photopic and scotopic conditions in AO.

Figure 4: (a and b) Chorioretinal lesions hypofluorescent in early stages and hyperfluorescent in late stages

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The diagnosis of BCR was established by initiating treatment with 60 mg/day of oral prednisone. After 2 months without improvement, we introduced cyclosporin A 400 mg/12 h. The patient reported very poor tolerance and adherence to treatment, so we decided to switch to azathioprine (Inmurel ^®) 50 mg/day. After 3 years of inactivity, the patient is asymptomatic and clinically stable.

Case 3

A 52-year-old male patient presented to the hospital manifesting photophobia and blurred vision of 3 years of evolution.

The BCVA was 0.6 in his RE and 0.8 in his LE. A mild Tyndall was observed in the anterior chamber. Funduscopically, we observed vitritis +, mild papillary edema, epiretinal membrane in both macules, demonstrated by macular OCT, and creamy lesions in the posterior pole. On FA, multiple hypofluorescent foci were noted in the posterior pole and nasal retina with mild late staining and peripheral vasculitis in BE without ischemia. Autofluorescence showed more numerous juxtapapillary and nasal hypoautofluorescent lesions in the RE [Figure 5]a and [Figure 5]b. In complementary studies, only HLA A29 was positive. The EOG and ERG showed a severe and bilateral affectation of the response in photopic and scotopic conditions.

Figure 5: (a and b) Juxtapapillary and nasal hypoautofluorescent lesions

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The patient was diagnosed with evolved BCR, so we decided to start treatment directly with 250 mg every 12 h of mycophenolate (Cellcept ^®). In the absence of improvement, we increased the dose to 2 g daily.

Discussion

BCR is a recurrent disease that has associated progressive visual impairment due to its anatomical and functional complications. Regarding BCR, there is no consensus on which drug is appropriate to carry out the treatment or what the duration of the treatment should be.^[6] Some authors, like Gasch et al., expose in their works that up to 20% of the cases are self-limited and they reach a complete remission without treatment.^[4]

In clinical practice, systemic corticosteroids are used as initial treatment or as rescue therapy when acute disease outbreaks appear. However, their most widespread use is when they are associated with systemic immunomodulators at the beginning, until they begin to make effect. The administration of periocular corticosteroids or intravitreal implants (Ozurdex ^® and Iluvien ^®) is reserved for cases of acute or refractory macular edema.

Among the options of immunomodulatory drugs are antimetabolites, such as methotrexate and mycophenolate mofetil or calcineurin inhibitors, such as cyclosporin A, which has become very popular in recent years in the treatment of noninfectious uveitis. All of them can be used in isolation or in association.

The evidence supporting the use of biological drugs in this disease is very limited. Sobrin et al. have described the use of daclizumab, a humanized monoclonal antibody directed against interleukin (IL)-2 receptor, reaching stability with complete resolution of vitritis and improvement in VA of BE in seven of eight patients.^[7] Regarding the use of the anti-tumor necrosis factor infliximab agent, in the series of Artornsombudh et al., six patients had to leave the treatment due to the appearance of side effects.^[8] On the other hand, in the series collected by Mesquida et al., a recent drug, tocilizumab, a humanized monoclonal antibody directed against the IL-6 receptor, has managed to control inflammation and resolve refractory macular edema secondary to BCR in six eyes of three patients being well tolerated and without adverse effects described.^[9]

In spite of everything, BCR is a chronic and complex disease, with multiple relapses, with a progressive loss of VA and long-term structural and functional complications regardless of whether the patient is treated with corticotherapy or immunomodulatory drugs.

Conclusions

Taking as reference the clinical cases of our series and given the visual prognosis and the uncertain natural history of the disease, the early introduction of immunomodulatory drugs for a prolonged period with a close monitoring is recommended. In fact, several studies have shown that the preservation of a good vision, the anatomical and functional integrity of the retina, as well as achieving long-term remissions are possible in those patients diagnosed with BCR and managed according to these premises.^[10]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Minos E, Barry RJ, Southworth S, Folkard A, Murray PI, Duker JS, et al. Birdshot chorioretinopathy: Current knowledge and new concepts in pathophysiology, diagnosis, monitoring and treatment. Orphanet J Rare Dis 2016;11:61.
2.	Levy-Clarke G, Jabs DA, Read RW, Rosenbaum JT, Vitale A, Van Gelder RN. Expert panel recommendations for the use of anti-tumor necrosis factor biologic agents in patients with ocular inflammatory disorders. Ophthalmology 2014;121:785-96.e3.
3.	Shah KH, Levinson RD, Yu F, Goldhardt R, Gordon LK, Gonzales CR, et al. Birdshot chorioretinopathy. Surv Ophthalmol 2005;50:519-41.
4.	Gasch AT, Smith JA, Whitcup SM. Birdshot chorioretinopathy. Br J Ophthalmol 1999;83:241-9.
5.	Priem HA, Oosterhuis JA. Birdshot chorioretinopathy: Clinical characteristics and evolution. Br J Ophthalmol 1988;72:646-59.
6.	Becker MD, Wertheim MS, Smith JR, Rosenbaum JT. Long-term follow-up of patients with birdshot retinochoroidopathy treated with systemic immunosuppression. Ocul Immunol Inflamm 2005;13:289-93.
7.	Sobrin L, Huang JJ, Christen W, Kafkala C, Choopong P, Foster CS. Daclizumab for treatment of birdshot chorioretinopathy. Arch Ophthalmol 2008;126:186-91.
8.	Artornsombudh P, Gevorgyan O, Payal A, Siddique SS, Foster CS. Infliximab treatment of patients with birdshot retinochoroidopathy. Ophthalmology 2013;120:588-92.
9.	Mesquida M, Molins B, Llorenç V, Sainz de la Maza M, Adán A. Long-term effects of tocilizumab therapy for refractory uveitis-related macular edema. Ophthalmology 2014;121:2380-6.
10.	Multicenter Uveitis Steroid Treatment (MUST) Trial Research Group, Kempen JH, Altaweel MM, Holbrook JT, Jabs DA, Louis TA, et al. Randomized comparison of systemic anti-inflammatory therapy versus fluocinolone acetonide implant for intermediate, posterior, and panuveitis: The multicenter uveitis steroid treatment trial. Ophthalmology 2011;118:1916-26.