Intravenous immunoglobulin in recurrent-relapsing inflammatory optic neuropathy Hadas Stiebel-Kalish,* { MD; Naama Hammel,* { MD; Judith van Everdingen, { MD; Ruth Huna-Baron {§ ; Andrew G. Lee, " MD ABSTRACT N RE ´ SUME ´ Objective: Recurrent-relapsing inflammatory optic neuropathy, including chronic relapsing inflammatory and auto- immune optic neuropathies, is rare, but can cause severe visual loss. Long-term steroids may preserve vision, yet side effects are frequent. We describe our experience with intravenous immunoglobulins (IVIg). Design: A semi-prospective case series from 4 medical centres. Participants: Patients with steroid responsive recurrent-relapsing optic neuropathy. Methods: Semiprospective case series of IVIg treatment in steroid-responsive recurrent-relapsing optic neuropathy at 4 medical centres. Outcome measures included visual outcome; time to, and duration of, remission; duration of corticosteroid use; and adverse events. Results: Vision stabilized in all 6 patients treated with IVIg without steroids for extended periods of time. None improved and none worsened. One adverse event occurred during an IVIg infusion after 3 uneventful years of IVIg maintenance. Average steroid use prior to IVIg was 12 months. After IVIg treatment, 5/6 patients no longer required corticosteroids. Two patients experienced late relapses on IVIg, one of whom was treated with cyclosporine, the other with steroids. Conclusions: IVIg can be considered an effective steroid-sparing agent in selected cases with steroid-dependent recurrent-relapsing autoimmune optic neuropathy. Objet : La neuropathie optique inflammatoire re ´cidivante et re ´currente, comprenant les neuropathies optiques re ´cidivantes chroniques et auto-immunes, est rare, mais elle peut causer une se ´ve `re perte visuelle. Le recours a ` long terme de ste ´roı ¨des peut pre ´ server la vision, ne ´ anmoins les effets secondaires sont fre ´quents. Nous de ´crivons notre expe ´rience de l’utilisation de l’immunoglobuline intraveineuse (IgIV). Nature : E ´ tude semi-re ´trospective d’une se ´rie de cas de 4 centres me ´dicaux. Participants : Patients atteints d’une neuropathie optique re ´cidivante et re ´currente re ´pondant aux ste ´roı ¨des Me ´ thodes : E ´ tude semi-prospective d’une se ´ rie de cas de neuropathie optique inflammatoire re ´cidivante et re ´currente sensible aux ste ´roı ¨des, traite ´sa ` l’IgIV dans 4 centres me ´dicaux. La mesure des re ´ sultats comprenait le re ´sultat visuel : le moment et la dure ´e de la re ´mission; la dure ´e d’utilisation du corticoste ´roı ¨de; et les e ´ve ´nements inde ´sirables. Re ´ sultats : La vision s’est stabilise ´e chez les 6 patients traite ´s a ` l’IgIV sans ste ´roı ¨de pendant des pe ´riodes de temps prolonge ´es. Il n’y a eu ni ame ´lioration ni aggravation. Un e ´ve ´nement inde ´sirable s’est produit pendant une infusion d’IgIV apre `s 3 anne ´es de maintien de l’IgIV sans e ´ve ´nement. La dure ´e d’utilisation de ste ´roı ¨de avant l’IgIV a e ´te ´ de 12 mois en moyenne. Apre `s le traitement a ` l’IgIV, 5 patients sur 6 n’ont plus eu besoin de corticoste ´r- oı ¨des. Il y a eu re ´cidive tardive chez 2 patients sous IgIV; un a e ´te ´ traite ´ avec de la cyclosporine, l’autre, avec des ste ´roı ¨des. Conclusions : L’IgIV peut e ˆtre conside ´re ´e comme un agent efficace pour e ´pargner les ste ´roı ¨des dans des cas se ´lectifs de neuropathie optique auto-immune re ´currente et re ´cidivante de ´pendant de la ste ´roı ¨de. R ecurrent-relapsing inflammatory optic neuropathy includes autoimmune optic neuropathy (AON) and chronic-relapsing inflammatory optic neuropathy (CRION), and is a rare, often bilateral, typically steroid- responsive optic neuropathy. The typical course of AON was first described in the 1980’s by Kupersmith 1 and Dutton et al. 2 Although patients with AON have positive serum autoantibodies such as antinuclear antibodies, rheumatoid factor, anticardiolipin antibodies, and (or) a positive skin biopsy of nonlesional skin, the full criteria for rheumatologic disorders (systemic lupus erythematosis [SLE], Wegener’s vasculitis, rheumatoid arthritis) are not met. AON is a diagnosis of exclusion, distinguished from demyelinating, granulomatous, infectious, and other From *the Neuro-Ophthalmology Unit, Department of Ophthalmology, Rabin Medical Center, Petah Tikva, Israel; { Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; { Rotterdam Eye Hospital, Rotterdam, The Netherlands; § Neuro-Ophthalmology Service, Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Israel; and " Neuro-Ophthalmology Unit, Departments of Ophthalmology, Neurology, and Neurosurgery, and the H. Stanley Thompson Neuro-ophthalmology Clinic at The University of Iowa Hospitals and Clinics, Iowa City, Iowa Originally received Mar. 21, 2009. Revised July 31, 2009 Accepted for publication Aug. 10, 2009 Published online Jan. 13, 2010 Correspondence to Hadas Stiebel-Kalish, MD, Chief, Neuro- Ophthalmology Unit, Department of Ophthalmology, Rabin Medical Center, Petah Tikva 49100, Israel; kalishh@clalit.org.il This article has been peer-reviewed. Cet article a e ´te ´e ´value ´ par les pairs. Can J Ophthalmol 2010;45:71–5 doi:10.3129/i09-238 CAN J OPHTHALMOL—VOL. 45, NO. 1, 2010 71