Paraneoplastic and Autoimmune Retinopathies
Occasionally, retinal degeneration is a complication of cancer resulting from a paraneoplastic immunologic mechanism. BCSC Section 9, Uveitis and Ocular Inflammation, explains the role of the immune system in this process. The 2 main paraneoplastic retinopathy syndromes are (1) cancer-associated retinopathy (CAR; Fig 14-2) and (2) melanoma-associated retinopathy (MAR). The characteristic ERG findings in MAR are a preserved dark adapted a-wave followed by a strikingly reduced b-wave, resulting in an electronegative ERG, which is similar to the ERG findings in CSNB. Patients with MAR usually present with an acquired night blindness and shimmering photopsias.
A third entity, autoimmune retinopathy, refers to an acquired, presumed immunologically mediated, retinal degeneration that resembles paraneoplastic retinopathy but without any identifiable systemic malignancy.
Figure 14-2 Cancer-associated retinopathy (CAR). A, Color fundus photograph of CAR in a patient with ovarian carcinoma. Note the severe vascular attenuation without obvious pigmentary alterations. B, Fourier-domain cross-sectional optical coherence tomography (OCT) of an eye with CAR that shows hyperreflectivity (asterisk) and disruption (arrowheads) of the outer nuclear layer external limiting membrane as well as decreases in reflectivity of the inner segment ellipsoid zone (arrows).
(Part A courtesy of John R. Heckenlively, MD; part B used with permission from Mesiwala NK, Shemonski N, Sandrian MG, et al. Retinal imaging with en face and cross-sectional optical coherence tomography delineates outer retinal changes in cancer-associated retinopathy secondary to Merkel cell carcinoma. J Ophthalmic Inflamm Infect. 2015;5(1):53.)
Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a paraneoplastic syndrome characterized by multiple melanocytic lesions of the choroid that may be associated with rapidly progressive posterior subcapsular cataract, iris and ciliary body cysts, and exudative retinal detachment. BDUMP has been associated with various systemic malignancies (see Chapter 9). Acute exudative polymorphous vitelliform maculopathy, which is characterized by multiple waxing and waning subretinal vitelliform lesions, has been reported in association with metastatic cutaneous melanoma and other systemic malignancies.
Fox AR, Gordon LK, Heckenlively JR, et al. Consensus on the diagnosis and management of nonparaneoplastic autoimmune retinopathy using a modified Delphi approach. Am J Ophthalmol. 2016;168:183–190.
Grange L, Dalal M, Nussenblatt RB, Sen HN. Autoimmune retinopathy. Am J Ophthalmol. 2014;157(2):266–272.
Rahimy E, Sarraf D. Paraneoplastic and non-paraneoplastic retinopathy and optic neuropathy: evaluation and management. Surv Ophthalmol. 2013;58(5);430–458.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.