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要約 目的:再発を繰り返す活動性甲状腺眼症に対してテプロツムマブを投与し,眼球突出および複視の改善を認めた1例を報告する。
症例:甲状腺眼症に対して過去にステロイドパルス療法3回と放射線治療1回を受けた既往のある70代の女性。前回発作時のステロイドパルス後よりステロイド内服を継続していたが,X年1月に左眼痛と第一眼位での複視が出現した。来院時の臨床活動性スコア(CAS)は5点で症状の再燃を認め,甲状腺眼症再発と判断しステロイド内服を増量したが,ステロイドに抵抗性を示した。過去の累積ステロイド投与量が20gを超えており,既存治療の継続は困難と判断し,テプロツムマブ投与を開始した。
結果:テプロツムマブ8回投与後にCASは1点に改善し,眼球突出は左眼4mm,右眼3mm減少した。Hess赤緑試験において複視範囲の縮小と第一眼位での複視消失を認めた。MRIでは外眼筋の高信号は残存するものの,臨床症状は明らかに改善した。
結論:再発を繰り返す活動性甲状腺眼症に対して,テプロツムマブは特に眼球突出および複視改善に有効であり,長期ステロイド治療が困難な症例において有用な治療選択肢となる可能性がある。
Abstract Purpose:To report a case of recurrent active thyroid eye disease(TED) treated with teprotumumab, in which improvement of proptosis and diplopia was achieved.
Case:A woman in her 70s with TED had a history of three courses of intravenous steroid pulse therapy and one course of orbital radiation. On January of year X, she presented with left ocular pain and diplopia in the primary gaze position, although she had continued oral prednisolone treatment after her last acute episode. TED recurrence was confirmed with worsening Clinical Activity Score(CAS) of 5. Although oral corticosteroid dosage was increased, her symptoms did not improve. As her past cumulative steroid dosage exceeded 20 grams, continuing conventional therapy was not considered adequate. Therefore, teprotumumab treatment was initiated.
Results:After completing eight courses of teprotumumab, CAS improved to 1, and proptosis decreased by 4 mm in the left eye and by 3 mm in the right eye. The Hess red-green test demonstrated a reduction in the range of diplopia and resolution of diplopia in the primary gaze position. Although high signal intensity of the extraocular muscles persisted on MRI, clinical symptoms showed evident improvement.
Conclusion:Teprotumumab demonstrated high efficacy in improving proptosis and diplopia in cases of recurrent active TED, representing a promising therapeutic option for patients for whom long-term corticosteroid therapy is not adequate.

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