To assess the effect of maintenance therapy on visual outcomes in preventing recurrences one year after first onset in patients with aquaporin-4 antibody (AQP4Ab)-positive optic neuritis.
The medical charts of 56 patients with optic neuritis (22 with AQP4Ab-positive and 34 with AQP4Ab-negative) at Niigata University Medical and Dental Hospital were retrospectively analyzed. Clinical characteristics, including visual acuity and number of recurrences one year after first onset, were compared among patients who were AQP4Ab-positivie with and those without maintenance therapy such as oral prednisolone and azathioprine, as well as those who were AQP4Ab-negative.
The mean ages were 49.3 and 45.2 years in the AQP4Ab-positive and the AQP4Ab-negative groups. The female to male ratio was 21:1 and 18:16 in the two groups, respectively. Multiple between-group comparison showed a statistically significant difference in visual acuity one year after first onset between the AQP4Ab-positive without maintenance therapy group and the AQP4Ab-negative group (0.05 (median, same applies below) vs. 1.0, p < 0.01). There was also a statistically significant difference in the number of recurrences in the year after first onset between the AQP4Ab-positive with and without maintenance therapy groups (1 vs. 0, p < 0.01).
This study demonstrates that patients with AQP4Ab-positive optic neuritis without maintenance therapy had the poorest visual acuity and the most recurrences one year after first onset. These results indicate that reducing the number of recurrences with maintenance therapy could improve the visual outcomes in patients with AQP4Ab-positive optic neuritis.
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Lennon VA, Wingerchuk DM, Kryzer TJ, Pittock SJ, Lucchinetti CF, Fujihara K, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004;364:2106–12.
Lennon VA, Kryzer TJ, Pittock SJ, Verkman AS, Hinson SR. IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med. 2005;202:473–7.
Wingerchuk DM, Banwell B, Bennet JL, Cabre P, Carrol W, Chitnis T, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85:177–89.
Papais-Alvarenga RM, Carellos SC, Alvarenga MP, Holander C, Bichara RP, Thuler LC. Clinical course of optic neuritis in patients with relapsing neuromyelitis optica. Arch Ophthalmol. 2008;126:12–6.
Merle H, Olindo S, Bonnan M, Donnio A, Richer R, Smadja D, et al. Natural history of the visual impairment of relapsing neuromyelitis optica. Ophthalmology. 2007;114:810–5.
Pirko I, Blauwet LA, Lesnick TG, Weinshenker BG. The natural history of recurrent optic neuritis. Arch Neurol. 2004;61:1401–5.
Contentti EC, De Virgiliis M, Hryb JP, Gomez A, Morales S, Celso J, et al. Aquaporin-4 serostatus and visual outcomes in clinically isolated acute optic neuritis. J Neuroophthalmol. 2019;39:165–9.
Trebst C, Jarius S, Berthele A, Paul F, Schippling S, Wildemann B, et al. Update on the diagnosis and treatment of neuromyelitis optica: recommendations of the Neuromyelitis Optica Study Group (NEMOS). J Neurol. 2014;261:1–16.
Yamagami A, Wakakura M, Inoue K, Ishikawa H, Takahashi T, Tanaka K. Clinical characteristics of anti-aquaporin 4 antibody positive optic neuritis in Japan. Neuroophthalmology. 2018;43:71–80.
Palace J, Lin DY, Zeng D, Majed M, Elsone L, Hamid S, et al. Outcome prediction models in AQP4-IgG positive neuromyelitis optica spectrum disorders. Brain. 2019;145:1310–23.
Wakakura M, Minei-Higa R, Oono S, Matsui Y, Tabuchi A, Kani K, et al. Baseline features of idiopathic optic neuritis as determined by a multicenter treatment trial in Japan. Jpn J Ophthalmol. 1999;43:127–32.
Optic Neuritis Study Group. The clinical profile of optic neuritis. Experience of the Optic Neuritis Treatment Trial. Arch Ophthalmol. 1991;109:1673–8.
Ishikawa H, Kezuka T, Shikishima K, Yamagami A, Hiraoka M, Chuman H, et al. Epidemiologic and clinical characteristics of optic neuritis in Japan. Ophthalmology. 2019;126:1385–98.
Takagi M, Tanaka K, Suzuki T, Miki A, Nishizawa M, Abe H. Anti-aquaporin-4 antibody-positive optic neuritis. Acta Ophthalmol. 2009;70:2197–200.
The authors were motivated to delineate the clinical characteristics of patients with AQP4Ab-positive optic neuritis after the case reports published by Takagi et al.  Although Dr. Takagi passed away in December 2012, his report contributed much to this study. We would like to thank Editage (www.editage.com) for English language editing.
Conflicts of interest
S. Ueki, None; T. Hatase, None; M. Kiyokawa, None; I. Kawachi, None; E. Saji, None; O. Onodera, None; T. Fukuchi, None; H. Igarashi, None.
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Corresponding Author: Satoshi Ueki
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Ueki, S., Hatase, T., Kiyokawa, M. et al. Visual outcome of aquaporin-4 antibody-positive optic neuritis with maintenance therapy. Jpn J Ophthalmol 65, 699–703 (2021). https://doi.org/10.1007/s10384-021-00858-0
- Optic neuritis
- Anti-aquaporin-4 antibody
- Visual outcome
- Maintenance therapy