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
. 2016 May;28(3):228-35.
doi: 10.1097/BOR.0000000000000271.

Retinal vasculitis

James T Rosenbaum  1 , Cailin H Sibley, Phoebe Lin
Affiliations
Review

Retinal vasculitis

James T Rosenbaum et al. Curr Opin Rheumatol. 2016 May.

Abstract

Purpose of review: Ophthalmologists and rheumatologists frequently have a miscommunication among themselves, and as a result differ in their opinion for patients consulting them with retinal vasculitis. This report seeks to establish a common understanding of the term, retinal vasculitis, and to review recent studies on this diagnosis.

Recent findings: The genetic basis of some rare forms of retinal vascular disease has recently been described. Identified genes include CAPN5, TREX1, and TNFAIP3; Behçet's disease is a systemic illness that is very commonly associated with occlusive retinal vasculitis; retinal imaging, including fluorescein angiography and other newer imaging modalities, has proven crucial to the identification and characterization of retinal vasculitis and its complications; although monoclonal antibodies to interleukin-17A or interleukin-1 beta failed in trials for Behçet's disease, antibodies to TNF-alpha, either infliximab or adalimumab, have demonstrated consistent benefit in managing this disease. Interferon treatment and B-cell depletion therapy via rituximab may be beneficial in certain types of retinal vasculitis.

Summary: Retinal vasculitis is an important entity for rheumatologists to understand. Retinal vasculitis associated with Behçet's disease responds to monoclonal antibodies that neutralize TNF, but the many other forms of noninfectious retinal vasculitis may require alternate therapeutic management.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Fundus imaging, optical coherence tomography(OCT), and ultra widefield fluorescein angiography findings upon initial presentation of a 25 year-old Saudi male diagnosed with Behçet’s disease. Fundus imaging of the right (A) and left (B) eyes reveal multifocal retinal infiltrates (solid white arrows), cotton wool spots (red arrow), and intraretinal hemorrhages (dotted white arrow) in the right eye > left eye. OCT reveals cystoid macular edema and a serous retinal detachment in the right eye (C), and a normal left eye (D). Ultra wide field imaging demonstrates diffuse retinal vascular leakage in both eyes, greater in the right (E) than the left (F).
Figure 2
Figure 2
Improvement in fundus imaging, optical coherence tomography (OCT), and ultra widefield fluorescein angiography findings after treatment with prednisone, azathioprine, and infliximab in Behçet’s disease. Fundus imaging of the right (A) and left (B) eyes reveal resolution of intraretinal infilrates and hemorrhages. OCT reveals resolution of cystoid macular edema and serous retinal detachment in the right eye (C), and normal OCT on the left eye (D). ultra widefield imaging demonstrates residual retinal vascular leakage in the right eye (yellow arrows) (E) and no retinal vascular leakage in the left eye (F).
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Sigler EJ, Grosso A. Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis Syndrome. Retina. 2015 Epub ahead of print. - PubMed
    1. Westland T, Kamphuis S, van Rossum AM, Rothova A. Case report: an 11-year-old girl presenting with asymptomatic retinal vasculitis leading to diagnosis of HIV-1 infection. Aids. 2015;29(14):1901–1902. - PubMed
    1. Rosenbaum JT, Ku J, Ali A, Choi D, Suhler EB. Patients with retinal vasculitis rarely suffer from systemic vasculitis. Semin Arthritis Rheum. 2012;41(6):859–865. - PMC - PubMed
    1. Sandhu RK, Adams T, Sibley C, Suhler EB, Kim DH. Granulomatosis with Polyangiitis (Gpa) Presenting with Frosted Branch Angiitis. Retinal cases & brief reports. 2015 Epub ahead of print. - PubMed
    1. Najem CE, Yadav R, Carlson E. Successful use of Rituximab in a patient with recalcitrant multisystemic eosinophilic granulomatosis with polyangiitis. BMJ case reports. 2015:2015. - PMC - PubMed

Publication types

MeSH terms