title: Consensus on the Diagnosis and Management of Nonparaneoplastic Autoimmune Retinopathy Using a Modified Delphi Approach.
abstract: To develop diagnostic criteria for nonparaneoplastic autoimmune retinopathy (AIR) through expert panel consensus and to examine treatment patterns among clinical experts.
  
  
  
  Modified Delphi process.
  
  
  
  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.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, Maryland.
authors.forename: Austin R
authors.initials: AR
authors.lastname: Fox
authors.affiliation: Stein Eye Institute, David Geffen School of Medicine at the University of California, Los Angeles, Los Angeles, California.
authors.forename: Lynn K
authors.initials: LK
authors.lastname: Gordon
authors.affiliation: Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan.
authors.forename: John R
authors.initials: JR
authors.lastname: Heckenlively
authors.affiliation: Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida.
authors.forename: Janet L
authors.initials: JL
authors.lastname: Davis
authors.affiliation: Department of Ophthalmology, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Debra A
authors.initials: DA
authors.lastname: Goldstein
authors.affiliation: Cole Eye Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Careen Y
authors.initials: CY
authors.lastname: Lowder
authors.affiliation: Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, Maryland.
authors.forename: Robert B
authors.initials: RB
authors.lastname: Nussenblatt
authors.affiliation: Massachusetts Eye and Ear, Harvard Medical School, Boston, Massachusetts; Veterans Affairs Boston Healthcare System, Jamaica Plain, Massachusetts.
authors.forename: Nicholas J
authors.initials: NJ
authors.lastname: Butler
authors.affiliation: Department of Ophthalmology, George Washington University, Washington, DC.
authors.forename: Monica
authors.initials: M
authors.lastname: Dalal
authors.affiliation: Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan.
authors.forename: Thiran
authors.initials: T
authors.lastname: Jayasundera
authors.affiliation: Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Wendy M
authors.initials: WM
authors.lastname: Smith
authors.affiliation: Bristol Eye Hospital, University of Bristol, Bristol, United Kingdom.
authors.forename: Richard W
authors.initials: RW
authors.lastname: Lee
authors.affiliation: Ocular Immunology Laboratory, Casey Eye Institute, Oregon Health & Science University, Portland, Oregon.
authors.forename: Grazyna
authors.initials: G
authors.lastname: Adamus
authors.affiliation: Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, Maryland.
authors.forename: Chi-Chao
authors.initials: CC
authors.lastname: Chan
authors.affiliation: Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, Maryland.
authors.forename: John J
authors.initials: JJ
authors.lastname: Hooks
authors.affiliation: Department of Physiology & Pharmacology, Oregon Health & Science University, Portland, Oregon.
authors.forename: Catherine W
authors.initials: CW
authors.lastname: Morgans
authors.affiliation: Department of Pathology, Johns Hopkins University, School of Medicine, Baltimore, Maryland.
authors.forename: Barbara
authors.initials: B
authors.lastname: Detrick
authors.affiliation: Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, Maryland. Electronic address: senh@nei.nih.gov.
authors.forename: H Nida
authors.initials: HN
authors.lastname: Sen
mesh_terms: D001323:Autoantibodies / Q000097:blood; D001324:Autoantigens / Q000276:immunology; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology; D032921:Consensus; D003697:Delphi Technique; D006801:Humans; D059545:Paraneoplastic Syndromes, Ocular / Q000175:diagnosis; D012160:Retina / Q000276:immunology; D012164:Retinal Diseases / Q000175:diagnosis* / Q000276:immunology
chemical_list: D001323:Autoantibodies; D001324:Autoantigens