血栓性微血管病合并多发性肌炎/皮肌炎：三例报告及文献综述
Thrombotic Microangiopathy with Polymyositis/Dermatomyositis: Three Case Reports and a Literature Review.
作者信息
Yamada Saeko, Yamashita Hiroyuki, Nakano Masahiro, Hatano Hiroaki, Sasaki Toshiharu, Takahashi Yuko, Kaneko Hiroshi
机构信息
Division of Rheumatic Diseases, National Center for Global Health and Medicine, Japan.
出版信息
Intern Med. 2018;57(15):2259-2265. doi: 10.2169/internalmedicine.0512-17. Epub 2018 Aug 1.
Abstract
Thrombotic microangiopathies (TMAs) rarely accompany polymyositis/dermatomyositis. We treated three patients with dermatomyositis combined with TMA. A literature review identified 13 previously reported cases. Exacerbation of myositis at the time of the TMA onset was observed in 62.5% of all patients, suggesting that the TMA onset may be associated with autoantibody production. We also found that cases of TMA with polymyositis/dermatomyositis often had a poor treatment response rate (37.5%). Furthermore, even if treatment was effective, the mortality rate associated with subsequent complications was high, and the survival rate was low (18.8%). Therefore, careful attention should be paid to patient management after TMA treatment.
摘要
血栓性微血管病（TMA）很少与多发性肌炎/皮肌炎同时出现。我们治疗了3例皮肌炎合并TMA的患者。文献回顾发现了13例先前报道的病例。在所有患者中，62.5%的患者在TMA发病时出现了肌炎加重，这表明TMA的发病可能与自身抗体产生有关。我们还发现，TMA合并多发性肌炎/皮肌炎的病例治疗有效率往往较低（37.5%）。此外，即使治疗有效，后续并发症相关的死亡率也很高，生存率很低（18.8%）。因此，TMA治疗后应密切关注患者管理。
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