Consensus on the Diagnosis and Management of Nonparaneoplastic Autoimmune Retinopathy Using a Modified Delphi Approach




In a recent issue of the Journal , Fox and associates published a consensus statement on the diagnosis and management of autoimmune retinopathy (AIR). The authors should be commended on their historic international collaborative effort addressing this enigmatic disease. In their article, the authors have defined the presence of serum antiretinal antibodies (ARAs) as an essential diagnostic criterion for AIR. Recent papers have highlighted many of the uncertainties related to ARA testing. As these papers were not referenced or discussed by the authors, a brief discussion of the problems with ARA testing that were raised in these papers is warranted.


In addition to not being standardized, current ARA testing is not validated, without any data in the literature on the sensitivity, specificity, positive predictive value, or negative predictive value of test results. Although the authors have started a process to help develop a standardized assay, it would be helpful if validation of this assay were also performed.


A major problem with ARAs is the lack of evidence for pathogenicity in the majority of cases. Basic science data are needed to prove the pathogenicity of ARAs. The only ARA that has proven pathogenicity, by 3 independent laboratories, is anti-recoverin. Basic science data also exist for anti-α-enolase, but this has come from only 1 laboratory, and needs verification. The need for verification arises because anti-α-enolase, as well as other putative ARAs that have been reported, are targeting ubiquitous proteins. If these ARAs are truly pathogenic, then it is unclear why they are causing disease solely in the retina. The authors are addressing the need for a standardized assay in their efforts, but they have neglected the need for proving pathogenicity of these ARAs. If any international collaborative effort on ARAs is going to be successful, then proving pathogenicity of these ARAs must be paramount.


The authors have highlighted the fact that only 1 center performing commercial ARA testing has Clinical Laboratory Improvement Amendments (CLIA) program certification. However, CLIA approval simply refers to the practice of good laboratory techniques, and does not mean that the ARA testing being offered by this center is validated or clinically meaningful. It is unclear why the authors would highlight a single laboratory, when the testing from that laboratory has not been proven to be any more clinically valid or meaningful than testing from any other laboratory.


In summary, there is significant uncertainty regarding currently available ARA testing. The authors should be congratulated on their collaborative efforts to help clear up this ambiguity. In addition to standardization, validation of ARA assays will be required. Furthermore, data proving pathogenicity of these ARAs will be needed. Given the limitations with current ARA testing, it would seem that, aside from anti-recoverin, there is currently no clinical utility in ARA testing. The authors should comment on whether or not they feel that current ARA testing has any clinical usefulness. It would seem reasonable for the authors to advise clinicians against ordering ARA testing, with the exception of anti-recoverin, until their efforts have been successful.

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Jan 5, 2017 | Posted by in OPHTHALMOLOGY | Comments Off on Consensus on the Diagnosis and Management of Nonparaneoplastic Autoimmune Retinopathy Using a Modified Delphi Approach
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