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Birdshot Chorioretinopathy

Choroiditis


Presentation

  • 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]
Lymphadenopathy
  • At the age of 13, the patient was diagnosed with common variable immunodeficiency with splenomegaly, lymphadenopathy, and lymphoid interstitial pneumonia. Progressive generalized lymphadenopathy developed thereafter.[insights.ovid.com]
  • […] show an increased risk of mortality or infection. 39, 40 In uveitis studies, daclizumab treatment has not been associated with serious infection or death. 19 - 21, 41 The most common adverse effects reported are skin rashes, mild peripheral edema, and lymphadenopathy[doi.org]
Anemia
  • Two years after initial presentation, the patient developed normocytic anemia and high levels of inflammatory markers. Further workup yielded a diagnosis of WM.[ncbi.nlm.nih.gov]
Wound Infection
  • The daclizumab adverse effect profile in patients with renal allograft has been widely studied and does not differ from that of placebo except for an increased risk of cellulitis and wound infections. 18, 35 - 37 There have been no reports of increased[doi.org]
Gagging
  • Notably, some antigenic peptides, including the immunodominant epitope derived from HIV gag protein, have been shown to be solely dependent upon ERAP2 trimming ( 25 ).[doi.org]
Diarrhea
  • One patient developed a less serious adverse event, transient diarrhea, after 1 infusion, which did not recur.[doi.org]

Workup

  • 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]
Normocytic Anemia
  • Two years after initial presentation, the patient developed normocytic anemia and high levels of inflammatory markers. Further workup yielded a diagnosis of WM.[ncbi.nlm.nih.gov]
Lymphocytic Infiltrate
  • Abstract Birdshot chorioretinopathy (BCR), a chronic ocular inflammatory disease with characteristic choroidal lymphocytic infiltrates, has been strongly associated with human leukocyte antigen (HLA)-A29.[ncbi.nlm.nih.gov]
  • Separate from the melanoma, there were nodular lymphocytic infiltrates in the choroid and the ciliary body.[nature.com]

Treatment

  • 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

  • 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]
  • , treatment, prevention, prognosis, and additional useful information HERE.[dovemed.com]

Etiology

  • 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]
  • 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]
  • 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]
  • Labs were ordered to rule out infectious etiology, which were negative. HLA A,B, C were negative. All clinical findings were consistent with BCR despite being negative for HLA-A29 versus MC.[aaopt.org]

Epidemiology

  • 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]
  • Epidemiology of respiratory allergies: current data Rev Mal Respir 2000 ; 17 : 139-158 2010 Elsevier Masson SAS. All Rights Reserved.[em-consulte.com]
Sex distribution
Age distribution

Pathophysiology

  • Its pathophysiology is still poorly understood.[ncbi.nlm.nih.gov]
  • 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]
  • Offers the most comprehensive content available on retina, balancing the latest scientific research and clinical correlations, covering everything you need to know on retinal diagnosis, treatment, development, structure, function, and pathophysiology.[books.google.com]
  • Affected individuals are usually diagnosed around age 45, a common age of onset. [1] Pathophysiology Birdshot chorioretinopathy is an autoimmune disease associated with the Human leukocyte antigen haplotype (HLA)-A29 in 95 to 97.5% of the cases.[wikidoc.org]

Prevention

  • 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]
  • […] chorioretinopathy (birdshot uveitis) in the UK so that we can raise the profile of the eye condition, ensure appropriate funding is offered for those with birdshot and understand the incidence and prevalence so that useful research for new treatments or prevention[birdshot.org.uk]

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