Birdshot retinochoroidopathy: pathophysiology, diagnosis and treatment. (3rd April 2017)

• Record Type: Journal Article
• Authors: Small, Karen; Ferrara, Mariantonia; Schmidt, Alexander; Foster, C. Stephen
• Is Part Of: Expert opinion on orphan drugs. Volume 5:Number 4(2017:Apr.)
• Page Start: 321
• Page End: 329
• Publication Date: 2017-04-03
• Subjects: Birdshot retinochoroidopathy -- immunomodulatory therapy -- T-cell-mediated autoimmuny -- uveitis; Orphan drugs -- Periodicals; Rare diseases -- Periodicals; Chemotherapy -- Periodicals; 615.1
• DOI: 10.1080/21678707.2017.1300523
• Languages: English
• ISSNs: 2167-8707

Abstract

ABSTRACT: Introduction : Birdshot Retinochoroidopathy (BSRC) typically affects Caucasian middle-aged patients and is strongly associated with the human leukocyte antigen, HLA-A29. The pathogenesis is not completely understood, but retinal autoimmunity related to T cells are involved. Diagnosis is made from clinical exam and ocular imaging. Early initiation of corticosteroid-sparing systemic immunomodulatory therapy (IMT) with periodic diagnostic testing to direct therapy, is the current recommendation, as visual acuity is inadequate to monitor progression. Humanized recombinant monoclonal antibodies, as well as intravitreal and surgical steroid implants have been used effectively at least transiently in refractory patients. Areas covered : In this article, the authors review and present current literature on the pathophysiology, diagnosis and treatment of BSRC. Expert opinion : BSRC is a vision-threatening disease. Control of intraocular inflammation is essential for good visual outcomes. With the known complications of steroids and their transient effectiveness, these medications are best reserved for acute flare management. The authors' first therapeutic choice is the combination of cyclosporine and mycophenolate mofetil, with adjustments in medication and dosage based on inflammation status at follow-up evaluations. Once remission is achieved on medications, we strongly advocate to maintain the treatment for at least 2 years before tapering therapy.