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Graefe's Archive for Clinical and Experimental Ophthalmology

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01-03-2022 | Inflammatory Disorders

Autoimmune retinopathy: clinical, electrophysiological, and immunological features in nine patients with long-term follow-up

Authors: Khaled Safadi, Itay Chowers, Eyal Banin, Boris Rosin, Liran Tiosano, Radgonde Amer

Published in: Graefe's Archive for Clinical and Experimental Ophthalmology | Issue 3/2022

Abstract

Purpose

We aim to report on the clinical, imaging, immunological, and electrophysiological features of patients with autoimmune retinopathy (AIR) with long-term follow-up.

Methods

Single-center, retrospective study of a consecutive group of AIR patients treated in a tertiary academic medical center.

Results

Included were nine patients with a mean ± SD age at presentation of 65 ± 13 years and a median follow-up of 63 months (range 18–120). Five patients were known to have cancer. Median interval between onset of ocular symptoms and diagnosis of AIR was 36 months. Mean baseline and final LogMAR visual acuity were 0.72 ± 0.9 and 1.1 ± 1.2, respectively (p = 0.17). The most common funduscopic findings included optic atrophy and bone-spicule-like pigmentation. Thinning of the nerve fiber layer was the most frequent optical coherence tomographic abnormality. Electroretinographic (ERG) recordings demonstrated variably reduced cone- and rod-derived amplitudes in the majority of eyes at presentation. The most commonly detected anti-retinal antibody was anti-α-enolase. Treatment included immunomodulatory therapy and plasmapheresis. ERG tests showed stability in 64% of eyes throughout the treatment period.

Conclusion

This study highlights the importance of maintaining a high index of suspicion of AIR, particularly in late middle-aged and elderly patients with “unexplained” visual loss, in light of the non-specific posterior segment signs and the inconsistency of the routinely used ancillary tests.

Adamus G, Ren G, Weleber RG (2004) Autoantibodies against retinal proteins in paraneoplastic and autoimmune retinopathy. BMC Ophthalmol 4:1–9. https://doi.org/10.1186/1471-2415-4-5CrossRef

Adamus G (2003) Autoantibody-induced apoptosis as a possible mechanism of autoimmune retinopathy. Autoimmun Rev 2:63–68CrossRef

Adamus G, Champaigne R, Yang S (2020) Occurrence of major anti-retinal autoantibodies associated with paraneoplastic autoimmune retinopathy. Clin Immunol 210.https://doi.org/10.1016/j.clim.2019.108317

Heckenlively JR, Ferreyra HA (2008) Autoimmune retinopathy: a review and summary. Semin Immunopathol 30:127–134CrossRef

Grange L, Dalal M, Nussenblatt RB, Sen HN (2014) Autoimmune retinopathy. Am J Ophthalmol 157:266. https://doi.org/10.1016/j.ajo.2013.09.019CrossRefPubMed

Ferreyra HA, Jayasundera T, Khan NW et al (2009) Management of autoimmune retinopathies with immunosuppression. Arch Ophthalmol 127:390–397. https://doi.org/10.1001/archophthalmol.2009.24CrossRefPubMed

Jacobson DM, Thirkill CE, Tipping SJ (1990) A clinical triad to diagnose paraneoplastic retinopathy. Ann Neurol 28:162–167. https://doi.org/10.1002/ana.410280208CrossRefPubMed

Weleber RG, Watzke RC, Shults WT et al (2005) Clinical and electrophysiologic characterization of paraneoplastic and autoimmune retinopathies associated with antienolase antibodies. Am J Ophthalmol 139:780–794. https://doi.org/10.1016/j.ajo.2004.12.104CrossRefPubMed

Chan JW (2003) Paraneoplastic retinopathies and optic neuropathies. Surv Ophthalmol 48:12–38CrossRef

10.

Ohguro H, Yokoi Y, Ohguro I et al (2004) Clinical and immunologic aspects of cancer-associated retinopathy. Am J Ophthalmol 137:1117–1119. https://doi.org/10.1016/j.ajo.2004.01.010CrossRefPubMed

11.

Finn AP, Thomas AS, Stinnett SS et al (2018) The role of cystoid macular edema as a marker in the progression of non-paraneoplastic autoimmune retinopathy. Graefe’s Arch Clin Exp Ophthalmol 256:1867–1873. https://doi.org/10.1007/s00417-018-4084-8CrossRef

12.

Abazari A, Allam SS, Adamus G, Ghazi NG (2012) Optical coherence tomography findings in autoimmune retinopathy. Am J Ophthalmol 153.https://doi.org/10.1016/j.ajo.2011.09.012

13.

Pepple KL, Cusick M, Jaffe GJ, Mruthyunjaya P (2013) SD-OCT and autofluorescence characteristics of autoimmune retinopathy. Br J Ophthalmol 97:139–144CrossRef

14.

Tsang SH, Sharma T (2018) Autoimmune retinopathy. Advances in Experimental Medicine and Biology. Springer, New York LLC, pp 223–226

15.

Masaoka N, Emoto Y, Sasaoka A et al (1999) Fluorescein angiographic findings in a case of cancer-associated retinopathy. Retina 19:462–464. https://doi.org/10.1097/00006982-199919050-00022CrossRefPubMed

16.

Rahimy E, Sarraf D (2013) Paraneoplastic and non-paraneoplastic retinopathy and optic neuropathy: Evaluation and management. Surv Ophthalmol 58:430–458CrossRef

17.

Sawyer RA, Selhorst JB, Zimmerman LE, Hoyt WF (1976) Blindness caused by photoreceptor degeneration as a remote effect of cancer. Am J Ophthalmol 81:606–613. https://doi.org/10.1016/0002-9394(76)90125-2CrossRefPubMed

18.

Murphy MA, Thirkill CE, Hart WM (1997) Paraneoplastic retinopathy: a novel autoantibody reaction associated with small-cell lung carcinoma. J Neuroophthalmol 17:77–83PubMed

19.

Guy J, Aptsiauri N (1999) Treatment of paraneoplastic visual loss with intravenous immunoglobulin: Report of 3 cases. Arch Ophthalmol 117:471–477. https://doi.org/10.1001/archopht.117.4.471CrossRefPubMed

20.

Maleki A, Lamba N, Ma L et al (2017) Rituximab as a monotherapy or in combination therapy for the treatment of non-paraneoplastic autoimmune retinopathy. Clin Ophthalmol 11:377–385. https://doi.org/10.2147/OPTH.S120162CrossRefPubMedPubMedCentral

21.

Shildkrot Y, Sobrin L, Gragoudas ES (2011) Cancer-associated retinopathy: Update on pathogenesis and therapy. Semin Ophthalmol 26:321–328CrossRef

22.

Jabs DA, Nussenblatt RB, Rosenbaum JT et al (2005) Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop. Am J Ophthalmol 140:509–516CrossRef

23.

McCulloch DL, Marmor MF, Brigell MG et al (2015) ISCEV Standard for full-field clinical electroretinography (2015 update). Doc Ophthalmol 130:1–12CrossRef

24.

Igarashi N, Sawamura H, Kaburaki T, Aihara M (2019) Cancer-associated retinopathy developing after 10 years of complete breast cancer remission. Neuro-Ophthalmology 43:36–42. https://doi.org/10.1080/01658107.2018.1460761CrossRefPubMed

25.

Makiyama Y, Kikuchi T, Otani A et al (2013) Clinical and immunological characterization of paraneoplastic retinopathy. Investig Ophthalmol Vis Sci 54:5424–5431. https://doi.org/10.1167/iovs.13-11868CrossRef

26.

Adamus G, Aptsiauri N, Guy J et al (1996) The occurrence of serum autoantibodies against enolase in cancer-associated retinopathy. Clin Immunol Immunopathol 78:120–129. https://doi.org/10.1006/clin.1996.0021CrossRefPubMed

27.

Gitlits VM, Toh BH, Sentry JW (2001) Disease association, origin, and clinical relevance of autoantibodies to the glycolytic enzyme enolase. J Investig Med 49:138–145CrossRef

28.

Pancholi V (2001) Multifunctional α-enolase: Its role in diseases. Cell Mol Life Sci 58:902–920CrossRef

29.

Adamus G, Karren L (2009) Autoimmunity against carbonic anhydrase II affects retinal cell functions in autoimmune retinopathy. J Autoimmun 32:133–139. https://doi.org/10.1016/j.jaut.2009.02.001CrossRefPubMedPubMedCentral

30.

Adamus G, Yang S, Weleber RG (2016) Unique epitopes for carbonic anhydrase II autoantibodies related to autoimmune retinopathy and cancer-associated retinopathy. Exp Eye Res 147:161–168. https://doi.org/10.1016/j.exer.2016.05.012CrossRefPubMed

31.

Mantel I, Ramchand KV, Holder GE et al (2008) Macular and retinal dysfunction of unknown origin in adults with normal fundi: Evidence for an autoimmune pathophysiology. Exp Mol Pathol 84:90–101. https://doi.org/10.1016/j.yexmp.2007.10.006CrossRefPubMed

Title: Autoimmune retinopathy: clinical, electrophysiological, and immunological features in nine patients with long-term follow-up
Authors: Khaled Safadi
Itay Chowers
Eyal Banin
Boris Rosin
Liran Tiosano
Radgonde Amer
Publication date: 01-03-2022
Publisher: Springer Berlin Heidelberg
Published in: Graefe's Archive for Clinical and Experimental Ophthalmology / Issue 3/2022
Print ISSN: 0721-832X
Electronic ISSN: 1435-702X
DOI: https://doi.org/10.1007/s00417-021-05409-4

At a glance: The STEP trials

Springer Medicine

Autoimmune retinopathy: clinical, electrophysiological, and immunological features in nine patients with long-term follow-up

Abstract

Purpose

Methods

Results

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusion

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