抗アクアポリン4抗体陽性視神経脊髄炎スペクトラム障害の1例

A case of anti-aquaporin 4 antibody-positive neuromyelitis optica spectrum disorder with ophthalmologic-alone satralizumab induction

要約　目的：抗アクアポリン4（AQP4）抗体陽性視神経脊髄炎スペクトラム障害（NMOSD）を発症した60代女性に対し，眼科単独でサトラリズマブを導入した1例を経験したので報告する。

症例：68歳，女性。右眼の対光反射減弱と視力低下の精査加療目的に当科を紹介受診となった。当科初診時の視力は右（0.01），左（1.0）であった。右眼の対光反射遅延と相対的瞳孔求心路障害（RAPD）陽性，眼球運動時痛を認め，ゴールドマン視野検査で右眼は3象限を超える視野欠損を認めた。眼窩部MRI（STIR）では右視神経の軽度腫大と高信号を認め，造影MRIで右視神経に造影効果を認めた。第23病日に抗AQP4抗体陽性が判明し，急性期治療として2回のステロイドパルス療法と免疫吸着療法を施行した。後療法として低用量ステロイド内服を継続したが，右視力は初診時の（0.01）から（0.08）と改善に乏しかった。初診から18か月後にサトラリズマブを導入し，その後約1年が経過したが，再発は認めていない。

結論：抗AQP4抗体陽性NMOSDに対してサトラリズマブを導入した1例を経験した。NMOSDの再燃時やサトラリズマブの副作用出現時は迅速に対応できるよう，慎重に経過観察する必要がある。

Abstract　Purpose：Here we report a case of a woman in her 60s with anti-AQP4 antibody-positive neuromyelitis optica spectrum disorder（NMOSD）who received satralizumab as a single ophthalmic procedure.

Case：A 68-year-old woman was referred to our department for the close examination and treatment of a weakened light reflex and decreased visual acuity in her right eye. At the initial visit, the visual acuity was 0.01 in the right eye and 1.0 in the left eye, and she had delayed light reflex and positive relative afferent pupillary defect in the right eye, pain during eye movement, and a visual field defect exceeding three quadrants in the right eye on the Goldmann visual field test. Orbital short tau inversion recovery showed mild swelling and high signal intensity in the right optic nerve, while contrast-enhanced magnetic resonance imaging showed a contrast effect on the right optic nerve. On the 23rd day of her illness, she tested positive for anti-AQP4 antibody and was treated acutely with two steroid pulse therapies and immunoadsorption therapy. Although low-dose oral steroids were continued, visual acuity recovery in the right eye was poor, ranging from（0.01）at the time of the initial examination to（0.08）. Eighteen months after the initial diagnosis, satralizumab was introduced. Approximately 1 years passed but no relapse has been observed.

Conclusion：Here we described a case of satralizumab induction in a patient with anti-AQP4 antibody-positive NMOSD. This case demonstrates that patients should be carefully monitored for relapse of NMOSD and adverse effects of satralizumab so that they can be treated promptly.