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Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.

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Autoimmune retinopathy pathophysiology

Introduction

Overview

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

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

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.

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