Autoimmune Optic Neuropathy: Pathogenesis, Diagnosis, and Therapeutic Advances

Autoimmune optic neuropathy (AON) encompasses a heterogeneous group of immune-mediated optic nerve inflammatory disorders, characterized by progressive or recurrent visual loss with or without optic disc edema, and distinct autoantibody profiles and neuroimaging features. This review synthesizes current knowledge on AON, highlighting key advances in the discovery of disease-specific biomarkers [eg, aquaporin-4 (AQP4)-IgG for NMOSD, myelin oligodendrocyte glycoprotein (MOG)-IgG for MOG-associated disease (MOGAD), and glial fibrillary acidic protein (GFAP)-IgG for astrocytopathy], while neuroimaging and optical coherence tomography (OCT) aid subtype differentiation. Therapeutically, acute management relies on high-dose intravenous methylprednisolone (IVMP), with plasma exchange for steroid-refractory cases; long-term maintenance is subtype-tailored. Prognosis varies by subtype, with MOG-ON showing better recovery than AQP4-ON, and CRMP5-IgG-associated ON carrying poor outcomes. Even with breakthroughs in pathogenetic understanding and targeted treatments, challenges reinforce the importance of continued interdisciplinary research to optimize AON management.