Autoimmune Retinopathy: Current Concepts and Practices (An American Ophthalmological Society Thesis)

Abstract

Purpose: To elicit and evaluate opinions in the diagnosis and management of nonparaneoplastic autoimmune retinopathy (npAIR) among members of the American Uveitis Society (AUS) and to further the development of consensus and criteria in the diagnosis and management of npAIR. We hypothesize that despite lack of any clear guidelines, a general consensus in the clinical diagnosis and treatment of npAIR exists among uveitis experts.

Methods: A literature review was performed and a panel of uveitis experts was consulted to formulate a survey regarding the diagnosis and management of npAIR. An online survey of 10 questions was developed, and a link was distributed through the AUS membership discussion list. We defined "general consensus" as meaning that a majority (>50%) of the respondents provided the same answer to a question.

Results: Fifty-four members of the AUS responded to the survey. Thirty-eight members (70.4%) see one to three AIR patients per year. Greater than 50% consensus was reached on most items, particularly items relating to diagnostic features and tests (up to 96% consensus).

Conclusions: The diagnosis and management of npAIR is challenging, as standardized clinical and laboratory diagnostic criteria have yet to be established. The results of this study support the presence of consensus regarding certain aspects of npAIR, but also indicate the need for developing clear clinical diagnostic criteria and treatment guidelines.

FIGURE 1

Color fundus photograph of 39-year-old patient with npAIR. Note the mild attenuation of retinal vasculature, poor foveal reflex, mild optic nerve pallor, and an otherwise normal-appearing fundus. Patient’s visual symptoms began 6 years previously with scotomas and floaters in the left eye. Despite treatment with prednisone, mycophenolate mofetil, and cyclosporine, visual acuity gradually declined to 20/320 bilaterally. An electroretinogram shows significantly decreased cone and rod responses.

FIGURE 2

Immunohistochemistry performed using serum of patient with npAIR against fresh-frozen human donor retina shows staining at the outer nuclear layer. (Courtesy of Chi Chao Chan, MD)

FIGURE 3

Optical coherence tomography images of patients with npAIR with different severity. Top, Patient 1. Mild disruption at the level of photoreceptors in left eye (arrows). Patient had moderately decreased cone responses (50% to 60%). Bottom, Patient 2. Extensive loss of the photoreceptor layer in right eye of a patient with more severe disease.