Birdshot studies

Here are links to various scientific and research papers about the eye condition BSRC and various therapies which may be used to treat it.   They are organised in date order with the most recent at the top.  You can use the search function (right)  to locate papers by particular authors or on particular topics.

Review of Birdshot. Cyclosporine alone produces better outcomes than steroid aloneKayur H. Shah MD, Ralph D. Levinson MD, , Fei Yu PhD, Raquel Goldhardt MD, Lynn K. Gordon MD, PhD, Christine R. Gonzales MD, John R. Heckenlively MD, Peter J. Kappel MD and Gary N. Holland MD. US. 2007. This is a detailed article which covers the testing associated with monitoring Birdshot, as well as the history of the disease, symptoms and treatment. The authors state that cyclosporine is better at preserving vision than steroid taken without additional immunosuppression.

Comparison of Antimetabolite Therapies for Noninfectious Ocular Inflammation. Anat Galor MD, Henry A Leder MD, Douglas A Jabs MD, MBA, Sanjay D Kedhar MD, James P Dunn MD, George Peters III MD, Jennifer E Thorne MD, PhD.  US. 2007 These writers compared the effectiveness and side effect profiles of methotrexate, azathioprine (imuran) and mycophenolate mofetil (cellcept) in the treatment of 315 patients with noninfectious ocular inflammation. 128 patients with inflammatory eye disease were treated with methotrexate, 44 with azathioprine, (imuran) and 143 with mycophenolate (cellcept). Treatment success at the initial starting dose of the antimetabolite was achieved by 30% in the methotrexate group, 54% in the azathioprine (imuran) group and 51% in the mycophenolate (cellcept) group. After dose increase or addition of second immunosuppressive agents, the percentage of patients achieving treatment success was higher in the mycophenolate (cellcept) group than in the methotrexate and azathioprine (imuran) groups. The incidence of side effects was higher in the azathioprine (imuran) group compared to methotrexate and mycophenolate (cellcept) with more patients stopping  the drug due to side effects in the azathioprine (imuran) group. These writers conclude more patients experience treatment success when taking cellcept, than when taking methotrexate or imuran.

Long-Term Follow-Up of Patients with Birdshot Retinochoroidopathy Treated with Systemic Immunosuppression. Matthias D. Becker Michael S. Wertheim Justine R. Smith James T. Rosenbaum. US. 2005. These authors  reviewed the progress of  11 Birdshot patients  over 15 years. They treated five of these patients with azathioprine, (imuran) methotrexate, cyclosporine A, mycophenolate mofetil, (cellcept) and/or IvIg, as well as systemic steroid or periocular corticosteroid injections. In these patients, inflammation was reduced or stabilised. The writers conclude that even though there is still no agreed strategy for the management of Birdshot, that it is possible to use steroid sparing treatments and preserve vision.

Sirolimus for refractory uveitis. V A Shanmuganathan,E M Casely, D Raj, R J Powell, A Joseph, W M Amoaku and H S Dua.  UK . 2005. Sirolimus is developed from an antibiotic and like cyclosporine and tacrolimus, it inhibits T cells but in a slightly different way and also affects B cells. In this study, 8 patients were followed for 52 weeks. Disease was controlled in five and two remained in remission after all treatment ended. The three who failed treatment also suffered intolerable side effects.

Long term follow up of Birdshot patients treated with steroid sparing immunomodulatory therapy. Kiss S Ahmed M Letko E Foster CS. US. 2005. This paper reports on 35 Birdshot patients. 28 patients did not have their inflammation controlled at the point of referral and after referral, at some point during their six year follow up, all were treated with steroid sparing immunosuppression. 92.9% were treated with cyclosporine, 67.9% with mycophenolate mofetil, (cellcept) 17.9% with azathioprine, (imuran) 10.7% with oral methotrexate, and 7.1% with daclizumab (zenapax). Complications affecting the eye from Birdshot and/or corticosteroids were cataract (53.6%), cystoid macular oedema (35.7%), glaucoma (21.4%), epiretinal membrane (10.7%), and retinal detachment (3.6%). At the end of the follow up period, up to 89.3% of the patients’eyes had either the same or improved visual acuity. The 30-hertz flicker implicit time was prolonged in 58.3% of initial ERGs and in 62.5% of final ERGs. The bright scotopic amplitude was abnormal in 45.5% of initial and final ERGs. (These tests measure retinal function).

The writers conclude that long term preservation of vision is possible for Birdshot patients and prompt treatment with immunosuppressants offers the best hope of keeping retinal function.

The successful use of mycophenolate mofetil in a patient with active birdshot chorio-retinopathy refractory to azathioprine therapy: case reportVIANNA, RaulN. G.; AL-KHARUSI, Nadia and DESCHENES, Jean. Brazil. 2004. This paper describes the successful treatment of a Birdshot patient using mycophenolate mofetil (cellcept). The patient developed cystoid macular oedema and retinal vasculitis in both eyes and had 20/70 vision in both. She was initially treated with  high dose oral prednisone (steroid) and subtenon steroid injections in both eyes. However, a month later, her visual acuity had reduced to 20/100 and treatmentment with azathioprine (imuran) was started. Within two months, there was reduction of cystoid macular oedema in one eye and visual acuity improved to 20/25. The other eye also improved, but the cystoid macular oedema was not resolved and the visual acuity was 20/60. After a further four months, the oedema had resolved in the 20/25 eye, but not in the other, which suffered a further loss of visual acuity to 20/70. This eye received another steroid injection and after another four months, the cystoid macular oedema was resolved in both eyes and the patient had 20/20 and 20/30 vision. Four months after this, the cystoid macular oedema had recurred in one eye and visual acuity had reduced to 20/60 and 20/80. Since azathioprine (imuran) was not controlling the progression of the disease, treatment was changed to mycophenolate mofetil (cellcept). Within two months, visual acuity had improved to 20/20 and 20/30 and over the next three years of treatment, there was no recurrence of the cystoid macular oedema.

Birdshot retinochoroidopathy. A. Gasch, J Smith, and S. Whitcup. US. 1999. This is a lengthy paper which deals with diagnosis, treatment, tests to follow the progression of the disease and prognosis. The link will take you first to a page which gives further papers from the 20th century you may wish to read.  Comparing the information in these papers with the more recent papers should help us to appreciate the advances being made in the treatment of Birdshot, the rapidity of this progress and give us hope that our sight will be preserved, even though our prognosis, in the past, has been so grim. We should take comfort and hope from the idea that some of the best brains in the world are looking after our eyes.
Birdshot chorioretinopathy: clinical characteristics and evolution. H A Priem and J A Oosterhuis. Belguim. 1988. This is a comprehensive article which describes the clinical features of Birdshot and the criteria for diagnosis. The paper is based on the five year progression of the disease in 102 patients from 14 European eye clinics in five European countries. There is a table which charts the patients’ visual acuity at the end of the five years and states the treatment each received. Most received steroid only, a couple were treated with cyclosporine, one of these with cyclophosamide as well and a few with azathioprine (imuran). The visual acuity statistics tell us how important it is to receive effective treatment, if we wish to retain our sight for as long as possible.