Early recognition is crucial given risks for severe visual loss and because it is treatable with immunosuppressive treatment such as steroids[3] or B-cell depleting therapy.
[1] Given that CRION is responsive to immunosuppressive treatment, it was presumed to be immune-mediated,[3] but this was uncertain as at the time there were no known associated autoimmune antibodies.
[1] A chest X-ray or CT scan should be ordered if granulomatous optic neuropathy caused by sarcoidosis is suspected.
[3] Magnetic resonance imaging can capture optic nerve inflammation, but this finding is not present in all patients,[1][3][13] Diffusion tensor imaging has been shown to detect widespread white matter abnormalities in CRION patients with normal MRI findings.
[14] Five diagnostic criteria had been proposed in 2014:[3] CRION has been included as a subtype in a 2022 international consensus classification of optic neuritis.
[2] Treatment consists of three phases of immunotherapy: Visual acuity is dramatically worse with CRION than other forms of optic neuritis.
[1] At times, patients obtain complete restoration of vision, although exact success rates are unknown.
[3] The disease is noted to occur worldwide and across many ethnicities, with reported cases in all continents except Africa and Australia.