トシリズマブにより肉眼的血尿が改善したAAアミロイドーシス膀胱病変を伴う成人期若年性特発性関節炎の一例

• Published in: 臨床リウマチ Vol. 29; no. 1; pp. 45 - 51
• Main Authors: 橋本, 展洋, 林, 晃正, 小林, 久美子, 岩崎, 祐介, 小中, 八郎, 石津, 桃, 伏見, 博彰, 竹澤, 健太郎, 山田, 貴久, 加藤, 保宏, 藤原, 弘士
• Format: Journal Article
• Language: Japanese
• Published: 一般社団法人 日本臨床リウマチ学会 2017; The Japanese Society for Clinical Rheumatology and Related Research
• Subjects: AA amyloidosis; gross hematuria; juvenile idiopathic arthritis; tocilizumab; urinary bladder
• ISSN: 0914-8760; 2189-0595
• DOI: 10.14961/cra.29.45

    A 25-year-old woman with 11-year history of juvenile rheumatoid arthritis was admitted to our hospital due to pericardial and bilateral pleural effusion. Prednisolone 1mg/kg/day improved these symptoms and were gradually tapered. However, she developed gross hematuria that induced severe anemia required transfusion during the hospitalization. Biopsy specimens of the urinary bladder revealed amyloidosis and immunohistochemical staining of the specimens defined the process as amyloid AA. The amyloid deposits were also found in the kidney and heart. Tocilizumab, a humanized anti-interleukin-6 receptor antibody, was started at a dose of 8mg/kg every 4 weeks. Subsequently, gross hematuria disappeared quickly. Although AA amyloidosis of the gastrointestinal tract, kidney, and myocardium has already been reported to be improved by tocilizumab, this is the first report on improvement of AA amyloidosis of urinary bladder presenting gross hematuria by tocilizumab.    25歳女性．14歳で若年性特発性関節炎多関節型と診断された．25歳時に心嚢液貯留，両側胸水にて入院となる．大量ステロイド療法にてそれらは改善した．しかし，輸血を必要とする肉眼的血尿が出現し，膀胱生検にてAAアミロイドーシスの膀胱病変と診断された．トシリズマブによる治療を開始したところ，血尿は消失した．AAアミロイドーシスの膀胱病変に対するトシリズマブの効果を示した最初の症例として報告する．