Clinical characteristics, diagnostic criteria and therapeutic outcomes in autoimmune optic neuropathy.
OBJECTIVE
Autoimmune optic neuropathy (AON) is characterised by chronically progressive or recurrent visual loss associated with serological 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. The 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 12 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) and azathioprine/chlorambucil (n = 1), and one received multiple combinations of agents. Criteria for diagnosis are proposed.


CONCLUSIONS
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.
Frohman
L
L
Department of Ophthalmology and Visual Sciences, UMDNJ-New Jersey Medical School, Newark, NJ 07103, USA. frohman@umdnj.edu
Dellatorre
K
K
Turbin
R
R
Bielory
L
L
D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D001706:Biopsy; D002648:Child; D005189:False Positive Reactions; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009901:Optic Nerve Diseases / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D012008:Recurrence; D012189:Retrospective Studies; D012867:Skin / Q000473:pathology; D016896:Treatment Outcome; D014786:Vision Disorders / Q000209:etiology / Q000503:physiopathology; D014792:Visual Acuity / Q000187:drug effects; D055815:Young Adult
D001323:Autoantibodies; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents