To present a novel case of paracentral acute middle maculopathy in association with birdshot chorioretinopathy. Case report. A patient presented with decreased vision and findings of uveitis, vasculitis and a paracentral scotoma. [ncbi.nlm.nih.gov]

The major shortcomings are the omission of ICGA signs, which are present in 100% of cases 19, 23 ; the lack of any reference to the visual field changes that are present in almost 100% of cases at presentation, depending on the onset of symptoms 32, 33 [doi.org]

Further workup yielded a diagnosis of WM. His choroidal lesions were significantly reduced after treatment with rituximab and bendamustine. We report a case of WM masquerading as BCR. [ncbi.nlm.nih.gov]

Workup revealed a positive HLA-A29 and was negative for sarcoid, tuberculosis, and syphilis.Conclusion:Birdshot chorioretinopathy overwhelmingly affects non-Hispanic Caucasians, but there have been rare reported cases in other ethnicities including Hispanics [scholars.northwestern.edu]

EXPOSURES: Patients received no treatment, short-term ( 1 year) treatment including local or systemic corticosteroids, or long-term ( 1 year) treatment including systemic corticosteroids and second-line immunosuppressive agents. [ncbi.nlm.nih.gov]

Six patients in this study continue to receive daclizumab treatment on a regular basis. The appropriate length of treatment is unclear and may be indefinite. [doi.org]

Early introduction of corticosteroid-sparing immune modulatory treatment (cyclosporine, methotrexate, mycophenolate mofetil, azathioprine, tacrolimus, human immunoglobulin G) is advocated, as extended treatment is anticipated in most patients. [orpha.net]

Prognosis Preservation of central vision until late in the disease and induction of long-term remission are possible with treatment, however the long-term visual prognosis of this disorder remains guarded. [orpha.net]

Conclusion The visual prognosis of BCR in a spectrum of uveitis is poor, and the recommended therapeutic regimens have had no effect on long-term visual prognosis. New treatment strategies are needed for this blinding disorder. [aaojournal.org]

Etiology Etiology is unknown but an organ -specific T-cell - driven autoimmune process is implicated, with both choroid and retina being independent targets and sites of inflammation. [orpha.net]

Purpose: Birdshot retinochoroidopathy (BRC) is a rare uveitis syndrome of presumed autoimmune etiology. Therapy with systemic and periocular corticosteroids is of inconsistent efficacy, attendant with numerous potential long-term side effects. [doi.org]

Birdshot retinochoroidopathy (BRC) is a rare uveitis syndrome of presumed autoimmune etiology. Therapy with systemic and periocular corticosteroids is of inconsistent efficacy, attendant with numerous potential long-term side effects. [ncbi.nlm.nih.gov]

Summary Epidemiology The disease is more common in North European descents with a female preponderance. It accounts for 6%-8% of cases of posterior uveitis. Prevalence in North Carolina is estimated to be 1/700,000. [orpha.net]

Discovery of HLA class I-specific killer cell immunoglobulin-like receptors (KIR) led to a series of epidemiological studies implicating KIR-HLA gene combinations in disease. [ncbi.nlm.nih.gov]

Keywords: chorioretinitis • clinical (human) or epidemiologic studies: outcomes/complications • uveitis-clinical/animal model 2007, The Association for Research in Vision and Ophthalmology, Inc., all rights reserved. [iovs.arvojournals.org]

Its pathophysiology is still poorly understood. [ncbi.nlm.nih.gov]

Its pathophysiology is still poorly understood. BSCR is the strongest documented association between HLA and disease in humans, which… CONTINUE READING [semanticscholar.org]

Pathophysiology Focal Lymphocytic (CD8 T-cells) infiltration of the choroid and choroidal blood vessels and mild retinal infiltration. Symptoms of Birdshot Retinochoroidopathy Birdshot Retinochoroidopathy is typically bilateral but asymmetric. [webeyeclinic.com]

IMPORTANCE: Birdshot chorioretinopathy (BCR) is a bilateral posterior uveitis that typically requires aggressive therapy to prevent loss of vision. [ncbi.nlm.nih.gov]

[…] the development of sunset glow fundus. 27 The preponderant role of ICGA in BRC has been reported by several groups in Europe and recently also in the USA. 19, 28–30 Indeed, prevention of BRC fundus lesions requires early diagnosis, which is not possible [doi.org]

Prevention[1] Birdshot retinochoroidopathy (BSRC) cannot be prevented. Once the diagnosis is made, preventative measures revolve around early identification and treatment of vision-threatening complications. [patient.info]

Most patients require treatment, which typically involves immunosuppressive medications to prevent vision loss. Although some people eventually lose vision, others maintain or recover good vision with therapy. The cause of BSCR is unknown. [retinaconsultants.org]