血栓性微小血管障害症に腎クリーゼなど多彩な臓器病変を合併した抗U3RNP抗体陽性全身性強皮症の一例

• Published in: 臨床リウマチ Vol. 26; no. 4; pp. 310 - 316
• Main Authors: 原中, 美環, 石井, 宏治, 尾崎, 貴士, 鳥越, 雅隆, 前島, 圭佑, 今田, 千晴, 清永, 恭弘, 柴田, 洋孝
• Format: Journal Article
• Language: Japanese
• Published: 一般社団法人 日本臨床リウマチ学会 2014; The Japanese Society for Clinical Rheumatology and Related Research
• Subjects: alveolar hemorrhage; plasma exchange(PE); scleroderma renal crisis; systemic sclerosis (SSc); thrombotic microangiopathy(TMA)
• ISSN: 0914-8760; 2189-0595
• DOI: 10.14961/cra.26.310

    We observed an anti-U3RNP antibody-positive case of systemic sclerosis (SSc) who developed thrombotic microangiopathy (TMA) with renal crisis and various other critical organ involvements. A 59-year-old woman consulted our outpatient department with cold-induced discoloration of the fingers, and the follow-up was started for Raynaudʼs phenomenon and antinuclear antibody positivity in2011. In the following year, she was diagnosed as SSc, based on progressive dermal sclerosis and fingertip pitting scars. In April 2013, she developed TMA with renal crisis,and acute heart failure at the same time, and was admitted to our hospital with an emergency. Administration of the ACE inhibitor, hemodialysis, and plasma exchange were started and high blood pressure and cytopenia due to TMA were controlled well. Methylpredonisolone pulse therapy to address progressive severe myocardial dysfunction was accompanied by a marked response,however, this resulted in diffuse alveolar hemorrhage shortly after. The respiratory damage subsided through continuation of the above treatment and strict management of cardiorespiratory condition. With the intensive therapy, each state of the disease was improved and stabilized successfully. In contrast to what our patient showed, some studies have suggested Japanese SSc patients with anti-U3RNP antibody positivity tend to have less frequent and less severe internal organ dysfunction. Multiple life-threatening complications, which our case presented with, are considered quite rare especially in a Japanese case with this antibody positivity and are thus worth reporting.    症例は59歳女性．2012年に全身性強皮症と診断された．翌年４月，血栓性微小血管障害症に強皮症腎クリーゼを併発し，更に急性心不全も伴っていた．ACE阻害薬の内服や血液透析，血漿交換などで加療され，また重症心筋障害にはステロイドパルスが奏功した．直後に肺胞出血を生じたが，厳格な呼吸循環管理と上記治療の継続で病状は安定した．重篤かつ多彩な病態に対し集学的治療で救命し得た全身性強皮症の一例を報告する．