Autoimmune retinopathy pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: M. Hassan, M.B.B.S

Overview

Autoimmune retinopathy (AIR) is an autoimmune retinal degenerative disease caused by serum autoantibodies cross reacting against the retinal, and retinal like antigens.

There are a significant number of anti-retinal antibodies that are associated with AIR, these include antibodies to anti-recoverin, anti-alpha-enolase, anti-transducin, anti-CAII, anti-arrestin, anti-rhodopsin, anti-Muller glial cells, anti-mitofilin, anti-tintin, anti-COX.  However, seronegative disease is also common.  AIR has been observed in patients with a history of autoimmune diseases and neoplastic diseases i.e melanoma.

Pathophysiology

  • Autoimmune retinopathy (AIR) is an autoimmune retinal degenerative disease caused by serum autoantibodies cross reacting against the retinal, and retinal like antigens. [1] [2]
  • There are a significant number of anti-retinal antibodies that are associated with AIR, these include antibodies to anti-recoverin, anti-alpha-enolase, anti-transducin, anti-CAII, anti-arrestin, anti-rhodopsin, anti-Muller glial cells, anti-mitofilin, anti-tintin, anti-COX.  However, seronegative disease is also common. 
  • Paraneoplastic autoimmune retinopathy (AIR) is triggered by molecular mimicry between tumor antigens and retinal proteins, and non-paraneoplastic AIR is triggered by cross reactivity between the viral or bacterial proteins and retinal proteins.
  • Both recoverin and α-enolase cause apoptosis of the retinal cells by cellular internalization via cascade pathways and intracellular calcium influx.   
  • AIR has been observed in patients with a history of autoimmune diseases and neoplastic diseases i.e melanoma. [3]
Type of Autoimmune retinopathy Antibody specificity
Cancer-associated retinopathy (CAR) Anti-recoverin, Anti-alpha-enolase, Anti-transducin, Anti-carbonic anhydrase II
Melanoma-associated retinopathy (MAR) Anti-transducin, Anti-carbonic anhydrase II, Anti-arrestin, Anti-rhodopsin, Anti-Muller glial cells,

Anti-mitofilin (mitochondrial protein), Anti-tintin, Anti-COX (cytochrome c oxidase, assembly mitochondrial protein)

Non-paraneoplastic AIR (npAIR) Anti-alpha-enolase, Anti-transducin, Anti-carbonic anhydrase II

References

  1. Dutta Majumder P, Marchese A, Pichi F, Garg I, Agarwal A (2020). "An update on autoimmune retinopathy". Indian J Ophthalmol. 68 (9): 1829–1837. doi:10.4103/ijo.IJO_786_20. PMC 7690499 Check |pmc= value (help). PMID 32823399 Check |pmid= value (help).
  2. Choi EY, Kim M, Adamus G, Koh HJ, Lee SC (2016). "Non-Paraneoplastic Autoimmune Retinopathy: The First Case Report in Korea". Yonsei Med J. 57 (2): 527–31. doi:10.3349/ymj.2016.57.2.527. PMC 4740551. PMID 26847311.
  3. Canamary AM, Takahashi WY, Sallum JMF (2018). "Autoimmune retinopathy: A Review". Int J Retina Vitreous. 4: 1. doi:10.1186/s40942-017-0104-9. PMC 5759752. PMID 29340169.


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