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      Relapse of Birdshot Uveitis after Stopping Immunosuppressive Treatment and Starting Immune Checkpoint Inhibitors for Lung Cancer


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          A 56-year-old Caucasian woman with birdshot uveitis had to stop immunosuppressive treatment with adalimumab due to metastatic squamous lung carcinoma. She was subsequently treated with chemotherapy and pembrolizumab, an immune checkpoint inhibitor (ICI). After stopping adalimumab and starting pembrolizumab, the patient had an inflammatory relapse of birdshot uveitis with macular oedema. Birdshot uveitis is triggered by an unknown antigen presented on the HLA-A29 molecule which activates cytotoxic T-cells. Although immunosuppressive therapy effectively stabilizes birdshot uveitis, it might induce a higher risk of developing cancer. Treatment with ICIs, on the other hand, might exacerbate birdshot uveitis by increasing anti-tumoural immune reaction and inducing off-target autoimmunity.

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          Most cited references13

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          Pembrolizumab plus Chemotherapy for Squamous Non–Small-Cell Lung Cancer

          Standard first-line therapy for metastatic, squamous non-small-cell lung cancer (NSCLC) is platinum-based chemotherapy or pembrolizumab (for patients with programmed death ligand 1 [PD-L1] expression on ≥50% of tumor cells). More recently, pembrolizumab plus chemotherapy was shown to significantly prolong overall survival among patients with nonsquamous NSCLC.
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            Birdshot chorioretinopathy.

            Birdshot chorioretinopathy is a well-known, yet poorly understood, form of posterior uveitis, characterized by multiple, distinctive, hypopigmented choroidal lesions, and strongly associated with human leukocyte antigen (HLA)-A29. We reviewed all English language publications regarding birdshot chorioretinopathy and performed analyses of combined patient data taken from these articles. The mean age at presentation was 53 years, with a slight female predominance (54.1%). At least 95.7% of reported patients have been HLA-A29-positive. Blurring of vision and floaters are the most prevalent presenting complaints, even in patients with visual acuity of 20/20 or better in both eyes. Birdshot chorioretinopathy is a slowly progressive disease with profound dysfunction of vision that may not be reflected in Snellen visual acuity. Two or more lines of Snellen visual acuity were lost in approximately 20% of eyes over a median follow-up of 3.5 years; macular edema was the most common cause of reduced visual acuity. Overall, patients had a slow decline in visual acuity, despite the fact that nearly all were treated with anti-inflammatory therapies. Final visual acuity in the better eye was 20/40 or better in 75.1% of patients and 20/200 or worse in 9.8% of patients. Oral corticosteroids and cyclosporine were the most commonly used medications. Using a regression model, patients in the literature that have been treated with cyclosporine alone had better final visual acuity than patients treated with oral corticosteroids alone. Further study is needed to determine the optimal methods for treating and monitoring patients with birdshot chorioretinopathy.
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              • Abstract: found
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              Birdshot chorioretinopathy: current knowledge and new concepts in pathophysiology, diagnosis, monitoring and treatment

              Birdshot chorioretinopathy (BCR) is a rare form of chronic, bilateral, posterior uveitis with a distinctive clinical phenotype, and a strong association with HLA-A29. It predominantly affects people in middle age. Given its rarity, patients often encounter delays in diagnosis leading to delays in adequate treatment, and thus risking significant visual loss. Recent advances have helped increase our understanding of the underlying autoimmune mechanisms involved in disease pathogenesis, and new diagnostic approaches such as multimodality imaging have improved our ability to both diagnose and monitor disease activity. Whilst traditional immunosuppressants may be effective in BCR, increased understanding of immune pathways is enabling development of newer treatment modalities, offering the potential for targeted modulation of immune mediators. In this review, we will discuss current understanding of BCR and explore recent developments in diagnosis, monitoring and treatment of this disease. Synonyms for BCR: Birdshot chorioretinopathy, Birdshot retinochoroiditis, Birdshot retino-choroidopathy, Vitiliginous choroiditis. Orphanet number: ORPHA179 OMIM: 605808.

                Author and article information

                Case Rep Ophthalmol
                Case Rep Ophthalmol
                Case Reports in Ophthalmology
                S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.com )
                Sep-Dec 2022
                17 November 2022
                17 November 2022
                : 13
                : 3
                : 910-915
                Department of Ophthalmology, ZNA Middelheim, Antwerp, Belgium
                Author notes
                *Mohammed A.M. Alsaddi, alsaddimohammed@ 123456gmail.com
                Copyright © 2022 by The Author(s). Published by S. Karger AG, Basel

                This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.

                : 19 May 2022
                : 11 August 2022
                : 2022
                Page count
                Figures: 1, Tables: 1, References: 13, Pages: 6
                No funding or financial support.
                Case Report

                birdshot uveitis,hla-a29,immune checkpoint inhibitors,pembrolizumab


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