PT  - JOURNAL ARTICLE
AU  - Larry P Frohman
AU  - Kara Della Torre
AU  - Roger Turbin
AU  - Leonard Bielory
TI  - Clinical Characteristics, Diagnostic Criteria, And Therapeutic Outcomes In Autoimmune Optic Neuropathy
AID  - 10.1136/bjo.2009.159350
DP  - 2009 Jan 01
TA  - British Journal of Ophthalmology
PG  - bjo.2009.159350
4099  - http://bjo.bmj.com/content/early/2009/08/13/bjo.2009.159350.short
4100  - http://bjo.bmj.com/content/early/2009/08/13/bjo.2009.159350.full
AB  - Background/Aims: Autoimmune optic neuropathy (AON) is characterized by chronically progressive or recurrent visual loss associated with serologic or cutaneous evidence of autoimmunity, without a defined systemic autoimmune illness. It may improve with large doses of corticosteroids alone, or in combination with immunosuppressive agents. Our aim was to determine the relative effectiveness of various therapeutic regimens in AON. Methods: All patients in this study fulfilled these criteria: 1) Multiple attacks in one eye or attacks in both eyes (at least three total attacks) 2) A minimum of twelve months of neuro-ophthalmic follow-up 3) Serological abnormalities or skin biopsy changes consistent with AON 4) No diagnosis of a defined collagen vascular disease or neurological autoimmune disease throughout follow-up, with the exception of one patient, later shown to be shown to be neuromyelitis optic antibody positive. Results: Nine cases were found (female=7, male =2, ages 8-74). One case received corticosteroids alone, the others received corticosteroids in combination with methotrexate/gammaglobulin (n=1), methotrexate (n=1), gammaglobulin (n=1), chlorambucil (n=2), cyclophosphamide (n=1), azathioprine/chlorambucil (n=1), and one received multiple combinations of agents. Criteria for diagnosis are proposed. Conclusion: As AON is quite rare, no formal recommendation can be made regarding its best therapy, although there is a suggestion that chlorambucil, although potentially toxic, may yield long term remission.