Adalimumab Therapy for Refractory Birdshot Chorioretinopathy

Veld, Paulien I. Huis in het; Asten, Freekje van; Kuijpers, Robert W A M; Rothová, Aniki; Hoyng, Carel B.

doi:10.1097/iae.0000000000002281

Cited by 18 publications

(13 citation statements)

References 21 publications

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“…The amount of evidence available steadily increased and, nowadays, indicates that aggressive and sustained immunosuppressive therapy is needed for the long-term treatment of BRC [ 27 , 28 ]. There is no specific agent that can be recommended but most conventional immunosuppressive [ 29 , 30 , 31 ] as well as biological agents [ 32 , 33 , 34 ] have been found to be efficient. The approach by trial and error has become possible as indocyanine green angiography was found sensitive for the monitoring of activity [ 35 ] giving rapid information on the efficacy or lack of efficacy of an agent in any given case, allowing fine-tuning of treatment [ 36 ].…”

Section: Resultsmentioning

confidence: 99%

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HLA-A29 Birdshot Retinochoroiditis in Its 5th Decade: Selected Glimpses into the Intellectual Meanderings and Progresses in the Knowledge of a Long-Time Misunderstood Disease

et al. 2021

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The appraisal of HLA-A29 birdshot retinochoroiditis (BRC) was fraught with pitfalls and misunderstandings. Progress in investigational methods has led to better knowledge and management of the disease. Our aim was to assess some of the steps that have led to better characterisation of the clinical entity of BRC. We performed a literature search analysing the relevant progress in disease origin, investigational and imaging methods, clinicopathology and classification, diagnostic criteria and management. Following developments were judged essential in the better appraisal and understanding of the disease: (1) new immunopathological hypotheses regarding the role of endoplasmic reticulum peptidases, (2) the essential importance of HLA testing, (3) relevant imaging modalities among which indocyanine green angiography is crucial, (4) diagnostic criteria that allow early diagnosis and (5) need of an early prolonged, as well as aggressive treatment combining more than one immunosuppressive agent. Based on these findings it is now possible to better define BRC, an indolent however severe disease, unlike thought before, involving the choroidal stroma and the retina independently and concomitantly that can be diagnosed early thanks to indocyanine green angiography and should be treated early and relentlessly.

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Section: Resultsmentioning

confidence: 99%

“…Indeed, one approach may function for one patient and not for another. Biologic agents represent additional therapeutic possibilities and have been shown to be useful [ 32 , 33 , 34 ]. However, for these therapies also, only trial and error will guide the clinician.…”

Section: Managementmentioning

confidence: 99%

HLA-A29 Birdshot Retinochoroiditis in Its 5th Decade: Selected Glimpses into the Intellectual Meanderings and Progresses in the Knowledge of a Long-Time Misunderstood Disease

et al. 2021

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“…Adalimumab obtained FDA approval for intermediate and posterior non-infectious uveitis based on the results of the VISUAL trial, a multicenter randomized phase 3 study in which 30 out of the 229 patients included had a birdshot chorioretinopathy [ 80 ]. Adalimumab has also been shown to be effective in improving the visual acuity in a report of 19 patients with BSCR refractory to immunosuppressive drugs, but complete remission was rarely achieved [ 81 ].…”

Section: Treatmentmentioning

confidence: 99%

Birdshot Chorioretinopathy: A Review

Bousquet

Duraffour

Debillon

et al. 2022

JCM

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Birdshot chorioretinopathy (BSCR) is a bilateral chronic inflammation of the eye with no extraocular manifestations. BSCR affects middle-aged individuals from European descent and is strongly associated with the human leucocyte antigen (HLA)-A29 allele. The immune mechanisms involved are not fully understood, but recent advances have shown the role of Endoplasmic Reticulum Aminopeptidase 2 (ERAP2) in disease pathogenesis. Multimodal imaging, including fluorescein angiography, indocyanine angiography, fundus autofluorescence, and optical coherence tomography, are useful in confirming the diagnosis and monitoring disease activity. Visual field testing is also important to assess the disease progression. To date, there is no consensus for optimal treatment regimen and duration. Local and systemic corticosteroids can be used for short periods, but immunosuppressive or biological therapies are usually needed for the long-term management of the disease. Here, we will review publications focused on birdshot chorioretinopathy to give an update on the pathophysiology, the multimodal imaging, and the treatment of the disease.

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“…Here, it is good to consider that the cause of HLA-A29-dependent BU is most likely heterogenous and in some patients may be mediated by genetic susceptibility imprinted in ERAP genes, while in others ERAPs may be dysregulated by alternative mechanisms. For example, ERAP1 is tightly regulated by TNF-alpha, a pro-inflammatory cytokine that is increased in concentration in eye fluids of BU patients and blocking TNF-alpha by anti-TNF therapy alleviates severe symptoms of BU (67)(68)(69)(70). Polymorphisms linked to ERAP1 and ERAP2 genes are also associated with other HLA class I associated conditions that manifest with non-infectious uveitis, including HLA-B27-associated anterior uveitis and ankylosing spondylitis, or HLA-B51-associated Behcet's disease (71)(72)(73).…”

Section: The Erap1-erap2 Haplotype Links Bu To the Western-european Ancestrymentioning

confidence: 99%

HLA-A29 and Birdshot Uveitis: Further Down the Rabbit Hole

Kuiper

Venema

2020

Front. Immunol.

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HLA class I alleles constitute established risk factors for non-infectious uveitis and preemptive genotyping of HLA class I alleles is standard practice in the diagnostic work-up. The HLA-A29 serotype is indispensable to Birdshot Uveitis (BU) and renders this enigmatic eye condition a unique model to better understand how the antigen processing and presentation machinery contributes to non-infectious uveitis or chronic inflammatory conditions in general. This review will discuss salient points regarding the protein structure of HLA-A29 and how key amino acid positions impact the peptide binding preference and interaction with T cells. We discuss to what extent the risk genes ERAP1 and ERAP2 uniquely affect HLA-A29 and how the discovery of a HLA-A29-specific submotif may impact autoantigen discovery. We further provide a compelling argument to solve the long-standing question why BU only affects HLA-A29-positive individuals from Western-European ancestry by exploiting data from the 1000 Genomes Project. We combine novel insights from structural and immunopeptidomic studies and discuss the functional implications of genetic associations across the HLA class I antigen presentation pathway to refine the etiological basis of Birdshot Uveitis.

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Adalimumab Therapy for Refractory Birdshot Chorioretinopathy

Abstract: The results suggest that adalimumab is effective at improving visual acuity and at tapering concomitant immunomodulatory therapy, in patients with refractory birdshot chorioretinopathy. However, complete remission is rarely achieved.

Cited by 18 publications

References 21 publications

HLA-A29 Birdshot Retinochoroiditis in Its 5th Decade: Selected Glimpses into the Intellectual Meanderings and Progresses in the Knowledge of a Long-Time Misunderstood Disease

HLA-A29 Birdshot Retinochoroiditis in Its 5th Decade: Selected Glimpses into the Intellectual Meanderings and Progresses in the Knowledge of a Long-Time Misunderstood Disease

Birdshot Chorioretinopathy: A Review

HLA-A29 and Birdshot Uveitis: Further Down the Rabbit Hole

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