TABLE 1.

Criteria and Tests for the Diagnosis of Autoimmune Retinopathy (AIR)

Diagnostic Criteria for AIRa
Essential Diagnostic Criteria	Supportive Diagnostic Criteria
No apparent cause responsible for visual function abnormalityb	Symptoms: Photopsias or scotomas or dychromatopsia or nyctalopia or photoaversion
ERG abnormality (with or without visual field abnormality)	Systemic autoimmune disease: Personal or Family History
Presence of serum antiretinal antibodies	Rapidity of onset of vision changee
Absence of fundus lesions and retinal degeneration or dystrophy that may explain visual function lossc
Absence of overt intraocular inflammationd
Core Diagnostic Testsf
Malignancy workup by appropriate physician	Fundus Autofluorescence (FAF)
Electroretinogram (ERG)	Optical Coherence Tomography (OCT)
Serum antiretinal antibody testing	Fluorescein Angiogram (FA)

^a. All Essential Diagnostic Criteria must be present and Supportive Diagnostic Criteria are not necessary to make the diagnosis of AIR

^b. Including no evidence of malignancy

^c. Absence of chorioretinal lesions (other than incidental/small peripheral benign degenerations such as pavingstone, lattice, etc., or old toxoplasmosis scar) or absence of retinal dystrophy, retinitis pigmentosa, or other hereditary retina vitreal disorders

^d. Less than 1+ intraocular cells or haze present

^e. Acute (0–3 months) or Subacute (3–6 months)

^f. Essential to the diagnosis of AIR and should be performed at the initial or first diagnostic evaluation when AIR is suspected