Autoimmune Retinopathy

Overview

Occurs when the immune system attacks the eye’s retina, causing inflammation. The disease is divided between paraneoplastic (immune system response to ​​a cancerous tumor) and non-paraneoplastic (immune system response to something other than a tumor). 

Paraneoplastic AIR can be further divided into subtypes:

  • Cancer-associated retinopathy (CAR)
  • Melanoma-associated retinopathy (MAR)

Common Symptoms

Common symptoms are night blindness, visual field defects, episodic flashing lights (photopsia), changes in the pigment of the retina, and retinal tissue loss.

Symptoms may differ based on which retinal cells are affected, as CAR affects both cones and rods while MAR affects just rods.

Symptoms specific to cones: 

photopsia, photosensitivity, hemeralopia, loss of color vision, diminished visual acuity, and diminished central vision.


Symptoms specifically related to rods:

Photopsia, nyctalopia, prolonged dark adaptation, and peripheral visual field loss.

Coexisting Diseases and Conditions

Non-paraneoplastic AIRs may coexist with Hashimoto’s thyroiditis, Grave’s disease, rheumatoid arthritis, multiple sclerosis, autoimmune hepatitis, and bullous pemphigoid.

Risk Factors and Prevalence

Risk factors differ based on the type of AIR present. MAR is more frequent in males, while females are more affected by CAR and non-paraneoplastic AIR. In addition, patients with non-paraneoplastic AIR are younger than those with the other forms. Individuals with a family history of autoimmune diseases may also be at greater risk for developing AIR.

Sources

  1. Article Sources and Footnotes
    1. Takahashi, W. Y., & Sallum, J. M. F. (2018, January 3). Autoimmune retinopathy: A review. International Journal of Retina and Vitreous. https://journalretinavitreous.biomedcentral.com/articles/10.1186/s40942-017-0104-9.

    2. Dutta Majumder, P., Marchese, A., Pichi, F., Garg, I., & Agarwal, A. (2020, September). An update on autoimmune retinopathy. Indian Journal of Ophthalmology . https://journals.lww.com/ijo/Fulltext/2020/68090/An_update_on_autoimmune_retinopathy.19.aspx

    3. Retina UK. (2019, November 14). Autoimmune retinopathy. Retina UK. https://retinauk.org.uk/about-inherited-sight-loss/types-of-inherited-sight-loss/autoimmune-retinopathy/.

    4. Khanna, S., Martins, A., Oakey, Z., & Mititelu, M. (2019). Non-paraneoplastic autoimmune retinopathy: multimodal testing characteristics of 13 cases. Journal of ophthalmic inflammation and infection, 9(1), 6. https://doi.org/10.1186/s12348-019-0171-1

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