Autoimmune retinopathy associated with monoclonal gammopathy of undetermined significance: a case report

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

Background

Monoclonal gammopathy of undetermined significance (MGUS) is a plasma cell dyscrasia and precursor to multiple myeloma. It has known ocular manifestations, but has not previously been shown to have an association with autoimmune retinopathy.

Case presentation

A 57 year-old female presented with 1 year of progressive, bilateral, peripheral vision loss, photopsias, and nyctalopia. Her fundus examination and extensive ancillary testing were concerning for hereditary versus autoimmune retinopathy. The patient was found to have anti-retinal antibodies against carbonic anhydrase II and enolase proteins with a negative genetic retinal dystrophy panel. Malignancy work-up was negative, but the patient was diagnosed with MGUS, a premalignant condition. The patient was treated with immunosuppressive therapies, with rituximab demonstrating the most robust therapeutic response with respect to patient symptoms and ophthalmic testing.

Conclusions

MGUS should be considered as a potential etiology of autoimmune retinopathy in patients without other autoimmune or malignant disease processes. Immunosuppressive therapy may be helpful in limiting disease progression, with rituximab showing efficacy in retinopathy refractory to other agents.

Related collections

Most cited references 16

Record: found
Abstract: found
Article: not found

Autoimmune retinopathy: a review and summary.

Gustavo Ferreyra, John R. Heckenlively (2008)

Three main forms of autoimmune retinopathy (AIR) have been identified over the last 15 years: cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), and nonneoplastic autoimmune retinopathy (npAIR). In this chapter, the term AIR will be used to encompass all three disorders where there is commonality to their features. Complicating the issue is that AIR can be a secondary complication of other conditions such as retinitis pigmentosa, ocular trauma, birdshot retinopathy, acute zonal occult outer retinopathy (AZOOR), or multiple evanescent white dot syndrome (MEWDS). The many forms of AIR tend to have common clinical features despite the fact that there has been no uniform set of anti-retinal antibodies circulating in these patients. Patients tend to have a wide variance of anti-retinal antibody activity often with three to six different antibodies found on immunoblots. Patients typically present with a sudden onset of photopsia, rapid visual loss, and abnormal electroretinograms (ERGs). Most patients have a panretinal degeneration without pigment deposits.

0 comments Cited 69 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Paraneoplastic and non-paraneoplastic retinopathy and optic neuropathy: evaluation and management.

Ehsan Rahimy, David Sarraf (2016)

Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. Timely recognition of one of these entities can facilitate early detection and treatment of an unsuspected, underlying malignancy, sometimes months before it would have otherwise presented, and gives the patient an increased chance at survival. We outline the clinical features, pathogenesis, and treatment strategies for the retinal- and optic nerve-based paraneoplastic syndromes: cancer-associated retinopathy; melanoma-associated retinopathy; paraneoplastic vitelliform maculopathy; bilateral diffuse uveal melanocytic proliferation; paraneoplastic optic neuropathy; and polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. Distinguishing these disorders from their non-paraneoplastic counterparts (e.g., autoimmune-related retinopathy and optic neuropathy, and acute zonal occult outer retinopathy) and determining appropriate systemic evaluation for the responsible tumor can be challenging. In addition, we discuss the utility and interpretation of autoantibody testing. Published by Elsevier Inc.

0 comments Cited 54 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Management of autoimmune retinopathies with immunosuppression.

K Jayasundera, Hong He, W Khan … (2009)

To report the results of treating autoimmune retinopathy (AIR) with immunosuppression therapy. Retrospective review of 30 consecutive patients with AIR followed for 3 to 89 months (median, 17 months) who were treated with immunosuppression (systemic or local). Subgroups were cancer-associated retinopathy (CAR), nonparaneoplastic AIR (npAIR), and npAIR with cystoid macular edema (npAIR/CME). Outcome measures were improvement of Snellen visual acuity by at least 2 lines, expansion of the visual field area by more than 25%, and resolution of CME. Overall, 21 of 30 patients (70%) showed improvement. All 6 CAR patients, 7 of 13 (54%) with npAIR, and 8 of 11 (73%) with npAIR/CME showed improvement. Five of 21 patients (24%) had improvement in visual acuity, 15 of 21 (71%) had expansion of visual field area, and 6 of 11 (55%) had resolution of CME. Twenty-six of 30 patients exhibited diffuse retinal atrophy without pigment deposits. An autoimmune family history was common in all the groups: npAIR, 69% (9 of 13); npAIR/CME, 64% (7 of 11); and CAR, 50% (3 of 6). Long-term treatment with immunosuppression resulted in clinical improvement in all subgroups of AIR. The most responsive subgroup was CAR; the least was npAIR. These results challenge the commonly held belief that AIR is untreatable.

0 comments Cited 52 times – based on 0 reviews      Review now

Bookmark

All references

Author and article information

Contributors

Emily A. Eton: etone@med.umich.edu

Gary Abrams: gabrams@med.wayne.edu

Naheed W. Khan: nwkhan@med.umich.edu

Abigail T. Fahim: ahteich@med.umich.edu

Journal

Journal ID (nlm-ta): BMC Ophthalmol

Journal ID (iso-abbrev): BMC Ophthalmol

Title: BMC Ophthalmology

Publisher: BioMed Central (London )

ISSN (Electronic): 1471-2415

Publication date (Electronic): 16 April 2020

Publication date PMC-release: 16 April 2020

Publication date Collection: 2020

Volume: 20

Electronic Location Identifier: 153

Affiliations

[1 ]GRID grid.214458.e, ISNI 0000000086837370, Kellogg Eye Center, , University of Michigan, ; 1000 Wall Street, Ann Arbor, MI 48105 USA

[2 ]GRID grid.254444.7, ISNI 0000 0001 1456 7807, Kresge Eye Institute, , Wayne State University, ; 4717 St. Antoine, Detroit, MI 48201 USA

Article

Publisher ID: 1423

DOI: 10.1186/s12886-020-01423-y

PMC ID: 7160953

PubMed ID: 32299429

SO-VID: 917e5856-e6a0-41b8-9455-09ffd281f268

License:

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

History

Date received : 21 May 2019

Date accepted : 6 April 2020

Custom metadata

ScienceOpen disciplines: Ophthalmology & Optometry

Keywords: autoimmune retinopathy,monoclonal gammopathy of undetermined significance,plasma cell dyscrasias

Data availability:

ScienceOpen disciplines: Ophthalmology & Optometry

Keywords: autoimmune retinopathy, monoclonal gammopathy of undetermined significance, plasma cell dyscrasias

Autoimmune retinopathy associated with monoclonal gammopathy of undetermined significance: a case report

Read this article at

Abstract

Background

Case presentation

Conclusions

Related collections

Karger: Ophthalmology

Most cited references 16

Autoimmune retinopathy: a review and summary.

Paraneoplastic and non-paraneoplastic retinopathy and optic neuropathy: evaluation and management.

Management of autoimmune retinopathies with immunosuppression.

Author and article information

Contributors

Journal

Affiliations

Article

History

Categories

Custom metadata

Comments

Comment on this article

Similar content 543

Cited by 3

Most referenced authors 186