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. 2016 Aug:168:183-190.
doi: 10.1016/j.ajo.2016.05.013. Epub 2016 May 20.

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

Austin R Fox  1 , Lynn K Gordon  2 , John R Heckenlively  3 , Janet L Davis  4 , Debra A Goldstein  5 , Careen Y Lowder  6 , Robert B Nussenblatt  1 , Nicholas J Butler  7 , Monica Dalal  8 , Thiran Jayasundera  3 , Wendy M Smith  9 , Richard W Lee  10 , Grazyna Adamus  11 , Chi-Chao Chan  1 , John J Hooks  1 , Catherine W Morgans  12 , Barbara Detrick  13 , H Nida Sen  14
Affiliations

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

Austin R Fox et al. Am J Ophthalmol. 2016 Aug.

Abstract

Purpose: To develop diagnostic criteria for nonparaneoplastic autoimmune retinopathy (AIR) through expert panel consensus and to examine treatment patterns among clinical experts.

Design: Modified Delphi process.

Methods: A survey of uveitis specialists in the American Uveitis Society, a face-to-face meeting (AIR Workshop) held at the National Eye Institute, and 2 iterations of expert panel surveys were used in a modified Delphi process. The expert panel consisted of 17 experts, including uveitis specialists and researchers with expertise in antiretinal antibody detection. Supermajority consensus was used and defined as 75% of experts in agreement.

Results: There was unanimous agreement among experts regarding the categorization of autoimmune retinopathies as nonparaneoplastic and paraneoplastic, including cancer-associated retinopathy and melanoma-associated retinopathy. Diagnostic criteria and tests essential to the diagnosis of nonparaneoplastic AIR and multiple supportive criteria reached consensus. For treatment, experts agreed that corticosteroids and conventional immunosuppressives should be used (prescribed) as first- or second-line treatments, though a consensus agreed that biologics and intravenous immunoglobulin were considered appropriate in the treatment of nonparaneoplastic AIR patients regardless of the stage of disease. Experts agreed that more evidence is needed to treat nonparaneoplastic AIR patients with long-term immunomodulatory therapy and that there is enough equipoise to justify randomized, placebo-controlled trials to determine if nonparaneoplastic AIR patients should be treated with long-term immunomodulatory therapy. Regarding antiretinal antibody detection, consensus agreed that a standardized assay system is needed to detect serum antiretinal antibodies. Consensus agreed that an ideal assay should have a 2-tier design and that Western blot and immunohistochemistry should be the methods used to identify antiretinal antibodies.

Conclusions: Consensus was achieved using a modified Delphi process to develop diagnostic criteria for nonparaneoplastic AIR. There is enough equipoise to justify randomized, placebo-controlled trials to determine whether patients with nonparaneoplastic AIR should be treated with long-term immunomodulatory therapy. Efforts to develop a standardized 2-tier assay system for the detection of antiretinal antibodies have been initiated as a result of this study.

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Figures

FIGURE 1
FIGURE 1. Consensus Development for the Diagnosis and Management of Autoimmune Retinopathy (AIR)
A modified Delphi process was utilized to develop consensus for the diagnosis and management of AIR and consisted of multiple rounds of surveys and the AIR Workshop. AUS: American Uveitis Society, AIR: Autoimmune retinopathy
FIGURE 2
FIGURE 2. Percent of Expert Survey Items Achieving Consensus for the Diagnosis and Management of Autoimmune Retinopathy (AIR)
Percent of items achieving consensusa between iteration 1 and 2 of the expert surveys remained stable and marginally increased overall (6.7%). a. Supermajority consensus is used for the clinical survey, while simple majority consensus was used for the basic laboratory survey due to the smaller number of items surveyed.
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Comment in

  • Consensus on the Diagnosis and Management of Nonparaneoplastic Autoimmune Retinopathy Using a Modified Delphi Approach.
    Forooghian F. Forooghian F. Am J Ophthalmol. 2016 Oct;170:241-242. doi: 10.1016/j.ajo.2016.06.046. Epub 2016 Aug 17. Am J Ophthalmol. 2016. PMID: 27544042 No abstract available.
  • Reply.
    Fox AR, Gordon LK, Heckenlively JR, Davis JL, Goldstein DA, Lowder CY, Nussenblatt RB, Butler NJ, Dalal M, Jayasundera T, Smith WM, Lee RW, Adamus G, Chan CC, Hooks JJ, Morgans CW, Detrick B, Sen HN. Fox AR, et al. Am J Ophthalmol. 2016 Oct;170:242-243. doi: 10.1016/j.ajo.2016.07.008. Epub 2016 Aug 25. Am J Ophthalmol. 2016. PMID: 27566926 No abstract available.

References

    1. Mizener JB, Kimura AE, Adamus G, Thirkill CE, Goeken JA, Kardon RH. Autoimmune retinopathy in the absence of cancer. Am J Ophthalmol. 1997;123(5):607–618. - PubMed
    1. Ferreyra HA, Jayasundera T, Khan NW, He S, Lu Y, Heckenlively JR. Management of autoimmune retinopathies with immunosuppression. Arch Ophthalmol. 2009;127(4):390–397. - PubMed
    1. Sen HN, Nussenblatt RB. Autoimmune retinopathies. In: Ryan, editor. Retina. 5th. London: Elsevier; 2013. pp. 1381–1389.
    1. Braithwaite T, Holder GE, Lee RW, Plant GT, Tufail A. Diagnostic features of the autoimmune retinopathies. Autoimmun Rev. 2014;13(4–5):534–538. - PubMed
    1. Larson TA, Gottlieb CC, Zein WM, et al. Autoimmune retinopathy: prognosis and treatment. Invest Ophthalmol Vis Sci. 2010;51 ARVO E-Abstract 6375.

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