A study of Irish patients with birdshot

This paper was given at the Prague Ocular Inflammation Society Congress in June 2009 by Dara Kilmartin Consultatant Ophthalmologists at Royal Victoria Hospital Dublin. It would seem to suggest that getting an early diagnosis is important.

Disease severity and outcomes with immunomodulation in Irish Patients with Birdshot Chorioretinoapthy. D.J. Kilmartin, A.C. Hogan, S. Jungkim, P. Kenna, Dept of Ophthalmology and Research Foundation, Royal Victoria Eye & Ear Hospital, Dublin, Ireland

Objective: To assess disease severity and outcomes in Irish BirdshotChorioretinopathy (BCR) patients when first receiving immunomodulatory therapy.

Methods: Retrospective case series of 11 BCR patients attendinga national tertiary uveitis clinic. Data collected included demographic details, functional assessment with fluorescein angiography, optical coherence tomography, Goldmann perimetry, electrophysiology, immunotherapy and clinical outcomes.

Results: Twenty two eyes of 11 patients, 7 female and 4 male, mean age 42 years with a mean follow up of 16 months (range 7-80) were assessed. At initial referral, Snellen visual acuity was 6/12 or better in 91% and 6/6 in 45% eyes; twenty eyes had active intraocular inflammation with choroiditis/vitritis (20), disc swelling (10), cystoid macular oedema (8). At baseline severe optic nerve dysfunction was evident in all eyes with reduced colour vision on Ishihara plate testing (14), blind spot enlargement (20) and paracentral scotomas (14) on Goldmann visual field, reduced amplitudes of pattern visual evoked responses (9) and advanced optic atrophy (2). Mean duration to tertiary referral was nine months (range 1-24) and 55% had a previous oral prednisolone pulse. During follow up patients were treated with oral prednisolone (11), mycophenolate mofetil (9, 2 intolerant) and tacrolimus (4). Disease relapses (n=19) occurred in 6 patients after achieving clinical inactivity, 58 % attributable to cystoid macular oedema. At latest follow up 73% of eyes had Snellen visual acuity 6/12 or better and were clinically inactive but all eyes had persistent optic nerve dysfunction.

Conclusions: Despite good presenting visual acuity, all Irish BCR
patients had significant optic nerve disease. Although treated
appropriately with combination immunosuppression, there was no
demonstrable improvement in optic nerve function due to delayed presentation.

2 thoughts on “A study of Irish patients with birdshot

  1. I have a family member, who was diagnosed with birshot chorioretinopathy, I would appreciate if you can let me know if any successful treatments are developed.

    • Hi John, I guess, from your email address, that you are in the US. The difficulty with Birdshot is that everyone seems to respond differently to different medication regimes, so the medication needs to be individually tailored to your relative. There is growing evidence that a regime of two different immunosuppressants and steroids is usually successful, but the type of immunousppressant will depend on what your relative can tolerate. There are a number of very eminent professors who understand Birdshot in the US. Two of the best known are Professor Stephen Foster at MERSI in Boston and Dr Michael Sampson in New York. Do e mail us at Birdshot@live.co.uk if you want more details or want to ask any more questions, and do register on our site at birdshot.org.uk if this is helpful. There is also a US based self help group, run by Dagmar and this can be accessed on http://bsrc.lefora.com/forum/category/bsrc-forum-topics/

      All the best

      Rea (and Annie)

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