Autoimmune retinopathy - a brief overview

ph02-1Autoimmune retinopathy is a very common type of eye condition which leads to blindness. Many of you may be unaware about the basics of the disease. So lets learn something more about the disease from a lecture session of Professor Wilson.

Wilson: Autoimmune retinopathy is a typical type of autoimmune disease which proactively damages different cells of the eye as for example the rods, cone s and retina cells.

Student: Why is it called autoimmune disease?

Wilson: Yes, I was coming to it. Actually you see, in this disease, antibodies are created against some of the retinal proteins, which in turn leads to degeneration of retinal cells.
Autoimmune retinal degeneration may occur in patients who present with sudden or, less commonly, subacute loss of vision of retinal origin, associated with an abnormal ERG, through the action of autoantibodies against retinal proteins. Often the patients are initially diagnosed with or suspected of having a paraneoplastic retinopathy (PR), such as cancer-associated retinopathy (CAR).
However, there is limited information on the occurrence, the specificity of autoantibodies in these patients, and their association with clinical symptoms.

Student: Is it caused only to those who have been contracted with cancer?

Wilson: Not exactly, but yes maximum cases, a study which reported about 61% of such cases where it has been seen that people affected with eye cancer are also associated with this eye condition.

Student: Sir, please clarify what are the symptomatic expression of this disease?

1471-2415-4-5-1-lWilson: The best-defined of this is associated with collapsin response-mediator protein-5 (CRMP-5)-immunoglobulin G (IgG) and manifests as bilateral optic neuritis with retinitis and vitritis. Now, as budding opthalmologist the very important part would be to make the proper diagnosis of this disease.
Fluorescein angiography is often performed to exclude other entities as potential causes of vision loss. Findings are usually normal, but, in occasional cases, fluorescein angiography may demonstrate mild peripheral vascular leakage consistent with vasculitis.
Thinning of the inner retinal layers in CAR has been demonstrated with optical coherence tomography (OCT).
The findings from full-field (Ganzfeld) ERG are almost always abnormal; specific findings depend on the predominance of cone versus rod dysfunction. Patients with CAR usually have absent cone responses. Findings in MAR include a markedly reduced or absent dark-adapted b wave, which indicates bipolar and Müller cell dysfunction.
Multifocal ERG (MERG) may be useful in select cases in which visual field loss is localized. In addition, some authors have used MERG to quantify the loss of electrical activity and to correlate this finding with results of Goldmann perimetry.

Student: What are the present treatments available?

Wilson: The overall prognosis of patients with PR is not good. Surgery, chemotherapy, and radiation therapy to treat the primary tumor do not appear to alter the visual prognosis. Various immunotherapies result in modest visual recovery in some cases.
Corticosteroids have been shown to decrease antibody titers in patients with CAR and may stabilize their vision, but they do not usually reverse vision loss. Anecdotal reports describe improvement in both CAR and MAR with high-dose intravenous methylprednisolone, plasmapheresis combined with steroids, or intravenous immunoglobulin (IVIG); however, the treatment results are largely disappointing. Espandar et al described a beneficial response in a patient with CAR treated with alemtuzumab, a monoclonal antibody that is used for the treatment of various B-cell mediated disorders. Calcium antagonists aimed at blocking antibody-mediated apoptosis were found to be protective against antirecoverin antibodies in an animal model, but the efficacy in humans has not yet been demonstrated.
In addition, azathioprine and gabapentin were reported to be of benefit in a patient with MAR.
Other research efforts involve activation of recoverin-specific antitumor cytotoxic T lymphocytes.

Student: One last question sir, who are mostly affected by this eye condition?

Wilson: O very important. Generally men contract this disease with a higher chance in age group over 57. Thank you class for your patience.

So, got what Mr. Wilson tried to explain all through his lecture session? I believe this would clear your doubt. For anything more and latest updates on this keep track with us.


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