This document, from Patient Plus, was published in 2007 and gives an overview of the diagnostic procedure and subsequent care a Birdshot patient may expect to receive in the UK.

The authors state that Birdshot is a progressive disease, that about 20% of patients will have a self limited course of this disease, and with few visual symptoms, require no treatment, while the rest will have chronic disease with periods of activity and remission. For the majority of patients, management of the disease is provided through cortisteroid (tablets and, if needed, injected into or around the eye) with cyclosporine or cytotoxic agents.

This paper describes a Birdshot patient followed clinically for 23 years. At presentation, in 1984, the patient’s vision was 20/20 in both eyes. The patient was initially treated with cortisteroid for a year with cyclosporine added at month nine and continued for two and a half years. At the end of this treatment, intraocular inflammation was controlled, but the patient had begun to experience kidney side effects from the cyclosporine and so treatment was stopped. There were mild episodes of inflammatory activity after treatment was ended, but systemic therapy was not restarted. By 1992, the patient’s visual acuity had reduced to 20/30 and 20/25, by 2001 colour vision was significantly affected and visual acuity was 20/60 and 20/40. The authors comment that there is lack of consensus about the drugs used in treatment and the duration of treatment, they state that vision drops late in the course of the disease, that retinal alterations and loss of retinal function progress despite minimal intraocular inflammation and make the point that since visual acuity remained stable till late in the course of disease, that vision

is not a good way to decide for treatment.

Describes the longterm progress of the disease.

This is a comprehensive over view of the treatment of uveitis in the UK written by a UK consultant. While it is not focused on Birdshot specifically, you will find details of the treatments available to us in the UK and their side effects. It contains a warning about imuran, for some patients, and ends with a five year plan.

O’NEIL M. BISCETTE, MD, MSCmpE • HOWARD F. FINE, MD, MHSc • THOMAS E. FLYNN, MD This is a comprehensive overview paper which covers the diagnosis of various forms of uveitis, provides a chart matching HLA tests to diseases and runs through the various groups of drugs used in treatment, explaining their actions and their side effects.

This is a very detailed paper and is perhaps best read when you feel you’ve grasped the basics about eyes in general, the disease and its treatment. If there is uncertainty about whether or not you actually have Birdshot, there is a section which deals with other uveitic eye diseases which may help you follow your specialist’s thinking about your own case. Like the other papers, this one describes the disease, what happens in the eye, treatments (with effective doses given), and makes some references to studies of others working in the same field. It’s a paper well worth dipping into when you are puzzled about something happening in your own eyes.

H A Priem and J A Oosterhuis. Belguim. 1988.

(You need to register to get the free adobe download).
If you want to see what our eyes look like inside, the images are here. 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. Scrolling to page 655, takes you to Table 9. If we reach a specialist, if we accept the modern therapies on offer, there is a good chance our eyes won’t end up on a table like this one.

By 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.

This paper delves into genetics and relates diseases to HLA markers. If you are puzzled about your HLA tests, this explains some of it and tells us how rare we and our eyes are. It also points out that animal studies are not always reliable – according to animal models, infliximab shoudn’t work. You need a medical/scientific mind to understand this paper.

Friederike Mackensen, Matthias D. Becker. 2006
Five patients with stubborn cystoid macular oedema and inactive uveitis at the time of treatment were given injections of avastin into their eyes. All five patients had a reduction in their macular oedema, four gained at least one additional line in visual acuity while the fifth’s remained the same. The authors note that longer follow up is required to see if the effect is transient or long lasting. More recent studies suggest that the effect is indeed transient in some patients, that repeated injections are required and it is emerging that the effect of repeated injections eventually wears off

Lucentis is a similar anti vegf injection to Avastin. It is given into the eye, and can be funded for macular degeneration patients in the UK. NHS Trusts will fund the first three injections and, at the moment, 2008, further injections are being funded by the drug company.

This link takes you to a ‘test your visual acuity’ online site. (Click the vision resources tab at the top of the web page.)

This link takes you to an online Amsler grid. It is useful for checking if you have cystoid macular oedema which is the
swelling of the retina and prevents us from seeing clearly, from reading easily, seeing faces clearly and seeing far into the distance. With slight cystoid macular edema, our central vision may still be very good, but when the lines on an Amsler grid look wavy, a little (or a lot) blurred or just plain odd, there is a good chance that we have cystoid macular oedema

If you need steroid or anti vegf injections into an eye, the thought of this procedure is far far worse than the actual injection. (Eye surgeons hardly hurt you at all, when compared with dentists and their needles).

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.

Adam Martidis, MD; Jay S. Duker, MD; Carmen A. Puliafito, MD. US. 2001.

This paper describes the resolution of cystoid macular oedema in two Birdshot patients through steroid injections into the eye. It contains OCT images which will help us understand our own OCT images should we have cystoid macular oedema. The authors point out the risks of these injections into the vitreous (jelly of the eye). The date of the paper, 2001, should be kept in mind. Since then, cyclosporine and/or cellcept has become accepted therapy for Birdshot and the anti vegf injections also can resolve CME. Sometimes an anti vegf injection (lucentis, macugen and avastin) is combined with steroid to enhance effect

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.

In this paper, the author also discusses two other studies giving statistics which demonstrate better visual outcomes in patients treated with cyclosporine or cellcept, rather than steroid alone.

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.

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.

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.

Kayur 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.