Purpose
To present a novel association of perivascular fundus autofluorescence abnormalities with the diagnosis of autoimmune retinopathy (AIR).
Methods
The retinal images of patients seen over the past two years (2010 to 2012) with the eventual diagnosis of AIR whose workup included fundus autofluorescence photographs were reviewed. The diagnosis of AIR was established based on a positive anti-retinal antibody titer and supported by compatible visual field and electroretinography studies. For all seven subjects meeting these criteria, a detailed chart review was performed. Demographic and clinical data were recorded, including gender, age, ethnicity, disease duration, visual acuity, and biomicroscopy and dilated fundus exam findings. Color fundus photographs, spectral domain optical coherence tomography (SD-OCT), and fundus autofluorescence imaging from the Spectralis HRA+OCT machine (Heidelberg Engineering, Heidelberg, Germany) and, when available, images from the P200CAF Wide-field-Autofluorescence scanning laser ophthalmoscope prototype (532-nm; Optos) were reviewed.
Results
Four patients with AIR had characteristic changes of perivascular hypoautofluorescence in very close association with the inner retinal vessels (see figure), thought to correspond to areas of retinal pigment epithelial (RPE) damage. Two patients had a less obvious pattern of perivascular hyper- and hypoautofluorescence, with hyperautofluorescence likely corresponding to active areas of RPE dysfunction. One patient had speckles of hyperautofluorescence distributed near but not adjacent to the vasculature as in the aforementioned cases. OCT confirmed the expected photoreceptor outer segment and RPE changes in areas of hyper- and hypoautofluorescence.
Conclusions
Characteristic perivascular autofluorescence abnormalities can be seen in AIR. Autofluorescence testing with both standard modalities and widefield imaging may have a role in the early detection and diagnosis of AIR, prompting testing for anti-retinal antibodies in the appropriate clinical context.
Keywords: 550 imaging/image analysis: clinical •
688 retina •
432 autoimmune disease