Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2020 Sep;68(9):1829-1837.
doi: 10.4103/ijo.IJO_786_20.

An update on autoimmune retinopathy

Affiliations
Review

An update on autoimmune retinopathy

Parthopratim Dutta Majumder et al. Indian J Ophthalmol. 2020 Sep.

Abstract

Autoimmune retinopathy (AIR) refers to a group of rare autoimmune retinal degenerative diseases presumably caused by cross-reactivity of serum autoantibodies against retinal antigens. The pathogenesis of AIR remains largely presumptive and there are a significant number of antiretinal antibodies that have been detected in association with AIR. The diagnosis of AIR is largely based on the demonstration of antiretinal antibodies in the serum along with suggestive clinical features and ancillary investigations. A high index of suspicion along with early diagnosis and treatment may play a critical role to lower the risk of irreversible immunological damage to the retinal cells in these patients. A multi-disciplinary approach for complete management and evaluation is helpful in such conditions. Various therapeutic options have been described for the treatment of AIR, though there is no consensus on standard treatment protocol.

Keywords: Autoimmune retinopathy; cancer-associated retinopathy; enolase; melanoma-associated retinopathy; recoverin.

PubMed Disclaimer

Conflict of interest statement

None

Figures

Figure 1
Figure 1
Graphical outline on proposed mechanism of autoimmune retinopathy involving anti-recovering antibody
Figure 2
Figure 2
Ultra-wide field fundus imaging of a patient with autoimmune retinopathy (non-paraneoplastic subtype) in a 45-year-old female (a and b). The fundus does not show any significant clinically visible changes. The fundus autofluorescence images (c and d) show very subtle changes especially in the nasal part of the left eye (d). There are stippled areas of hypo-autofluorescence corresponding to the retinal pigment epithelial damage
Figure 3
Figure 3
Spectral-domain optical coherence tomography (SD-OCT) of the same patient shows presence of intraretinal cystic spaces in the right eye (yellow arrows) in the perifoveal region. There is increase in the overall central macular thickness (a). The left eye shows mild increase in central retinal thickness (b). The visual field analysis (c and d) shows severe generalized field loss especially in the right eye and loss of nasal field in the left eye

References

    1. Sawyer RA, Selhorst JB, Zimmerman LE, Hoyt WF. Blindness caused by photoreceptor degeneration as a remote effect of cancer. Am J Ophthalmol. 1976;81:606–13. - PubMed
    1. Klingele TG, Burde RM, Rappazzo JA, Isserman MJ, Burgess D, Kantor O. Paraneoplastic retinopathy. J Clin Neuroophthalmol. 1984;4:239–45. - PubMed
    1. Gass J. Acute Vogt-Koyonagi-Harada-like Syndrome Occuring in a Patient with Metastatic Cutaneous. Melanoma Amsterdam, Netherland. 1984
    1. Thirkill CE, Roth AM, Keltner JL. Cancer-associated retinopathy. Arch Ophthalmol Chic Ill. 1960;1987(105):372–5. - PubMed
    1. Mizener JB, Kimura AE, Adamus G, Thirkill CE, Goeken JA, Kardon RH. Autoimmune retinopathy in the absence of cancer. Am J Ophthalmol. 1997;123:607–18. - PubMed