Chronic Relapsing Inflammatory Optic Neuropathy

Chronic Relapsing Inflammatory Optic Neuropathy (CRION) is a rare autoimmune optic neuritis, distinct from conditions like sarcoidosis, lupus, MS, or NMO. Diagnosed through MRI and exclusion of other causes, CRION treatment requires long-term immunosuppression, as glucocorticoid tapering often leads to relapse. Effective treatments include azathioprine, methotrexate, cyclophosphamide, and IVIG.

Overview of Chronic Relapsing Inflammatory Optic Neuropathy (CRION):

Chronic relapsing inflammatory optic neuropathy (CRION) is a rare, relapsing autoimmune optic neuritis in which no other known systemic disease could be identified including sarcoidosis, lupus, MS or neuromyelitis optica (NMO). (1)

Clinical Presentation and Diagnostic Approach in CRION:

The clinical feature is that of optic neuritis with enhancement of the optic nerve on MRI. Diagnostic work-up should exclude other systemic, metabolic or paraneoplastic causes of optic neuropathy, including a serum NMO immunoglobulin G (IgG) antibody test. (2)

Treatment Strategies for CRION:

For treatment of CRION, long term immunosuppressive agents are suggested. In contrast to patients with optic neuritis, tapering glucocorticoid therapy leads to a clinical relapse. In order to stay in remission, patients with CRION require chronic immunosuppressive agents, such as, azathioprine (2 mg/kg/day), methotrexate (7.5-10 orally mg once weekly), cyclophosphamide ( 750 -1.000 mg IV) or intravenous immune globulin (IVIG). (3, 4, 5)

References

1-Kidd D, Burton B, Plant GT, Graham EM. Chronic relapsing inflammatory optic neuropathy (CRION). Brain. 2003 Feb;126(Pt 2):276-84.

2-Petzold A, Plant GT. Chronic relapsing inflammatory optic neuropathy: a systematic review of 122 cases reported. J Neurol. 2014 Jan;261(1):17-26. Epub 2013 May 23.

3-Pacheco PA, Taylor SR, Cuchacovich MT, Diaz GV. Azathioprine in the management of autoimmune uveitis [published correction appears in Ocul Immunol Inflamm. 2009;17(6):438]. Ocul Immunol Inflamm. 2008;16(4):161-165.

4-Muñoz-Fernández S, García-Aparicio AM, Hidalgo MV, et al. Methotrexate: an option for preventing the recurrence of acute anterior uveitis. Eye (Lond). 2009;23(5):1130-1133.

5-Díaz-Llopis M, Gallego-Pinazo R, García-Delpech S, et al. General principles for the treatment of non-infectious uveitis. Inflamm Allergy Drug Targets. 2009;8(4):260-265.