July 2019
Volume 60, Issue 9
Free
ARVO Annual Meeting Abstract  |   July 2019
Birdshot Chorioretinitis: Treatment & Remission Outcomes
Author Affiliations & Notes
  • Eric L Crowell
    Ophthalmology, Johns Hopkins University, Baltimore, Maryland, United States
  • Richard France
    Ophthalmology, Icahn School of Medicine at Mount Sinai, New York City, New York, United States
  • Palak Majmudar
    Ophthalmology, Icahn School of Medicine at Mount Sinai, New York City, New York, United States
  • Douglas A Jabs
    Ophthalmology, Icahn School of Medicine at Mount Sinai, New York City, New York, United States
  • Jennifer E Thorne
    Ophthalmology, Johns Hopkins University, Baltimore, Maryland, United States
  • Footnotes
    Commercial Relationships   Eric Crowell, None; Richard France, None; Palak Majmudar, None; Douglas Jabs, None; Jennifer Thorne, None
  • Footnotes
    Support  Cross Family Birdshot Research Fund
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 3506. doi:
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    • Get Citation

      Eric L Crowell, Richard France, Palak Majmudar, Douglas A Jabs, Jennifer E Thorne; Birdshot Chorioretinitis: Treatment & Remission Outcomes. Invest. Ophthalmol. Vis. Sci. 2019;60(9):3506.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : To determine the incidence rates of corticosteroid tapering success after immunosuppressive drug therapy (IMT) has been initiated, corticosteroid discontinuation, drug-free remission, and relapse of birdshot chorioretinitis (BSCR).

Methods : All patients diagnosed with BSCR at The Wilmer Eye Institute Division of Ocular Immunology and Icahn School of Medicine at Mount Sinai Ocular Immunology Division prior to December 2017 were reviewed. Demographics, HLA-A29 status, laterality, visual acuity, diagnosis of macular edema and disease activity were recorded. Results of testing including electroretinogram, Goldman visual field, fluorescein angiography, and indocyanine green angiography were recorded. Therapeutic interventions with IMT and systemic, intra- and peri-ocular corticosteroid usage were recorded. Taper success was defined as the ability to taper corticosteroid to ≤7.5mg daily without recurrence of inflammation. Remission was defined according to SUN criteria. Recurrence was defined as the return of disease activity (new cell, macular edema, vasculitis, ICG hypocyanescence) and relapse as recurrence of inflammation during remission. Rates of corticosteroid tapering success and discontinuation, drug-free remission, and relapse of disease were calculated using Kaplan-Meier curves.

Results : One hundred seven patients were included in this study; 59.8% were women; 99.1% were white. All patients had bilateral disease and 97.8% of 91 patient tested were HLA-A29 positive. Corticosteroid tapering success was achieved in 57 patients (n=91, 62.6%) at a rate of 0.178 per person years (/PY; 95% CI 0.138/PY, 0.224/PY). Corticosteroids were discontinued in 54 patients (rate=0.183/PY; 95% CI 0.141/PY, 0.232/PY). Drug-free remission was achieved in 18 patients (rate=0.043/PY; 95% CI 0.026/PY, 0.067/PY). Recurrence of inflammation at any point during follow up occurred in 37 patients (rate=0.10/PY; 95% CI 0.072/PY, 0.136/PY). Relapse after remission was similar (rate=0.104/PY; 95% CI 0.056/PY, 0.171/PY).

Conclusions : Successful corticosteroid taper was achieved in the majority of BSCR patients. Remission appears to be possible in a small portion of patients but relapse after drug discontinuation is observed. Future analyses examining factors that increase the likelihood of remission and decrease the likelihood of relapse are needed.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.

 

Kaplan Meier plot of the proportion of people who achieve remission over time.

Kaplan Meier plot of the proportion of people who achieve remission over time.

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