Clinical characteristics, diagnostic criteria and therapeutic outcomes in autoimmune optic neuropathy.

作者:L Frohman, K Dellatorre, R Turbin, L Bielory
语言:Eng.
日期:2009-11-26
学报:The British journal of ophthalmology (1468-2079)
发行:Br J Ophthalmol. 2009 Dec;93(12):1660-6


摘要:



BACKGROUND/

AIMS:
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.

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.

版权:The British journal of ophthalmology

Department of Ophthalmology and Visual Sciences, UMDNJ-New Jersey Medical School, Newark, NJ 07103, USA. frohman umdnj.edu
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科目:Adolescent, Adult, Aged, Autoantibodies, Autoimmune Diseases, Biopsy, Child, False Positive Reactions, Female, Follow-Up Studies, Glucocorticoids, Humans, Immunoglobulins, Intravenous, Immunosuppressive Agents, Magnetic Resonance Imaging, Male, Middle Aged, Optic Nerve Diseases, Recurrence, Retrospective Studies, Skin, Treatment Outcome, Vision Disorders, Visual Acuity, Young Adult