Ohashi Naro, Yamamoto Tatsuo, Kanno Daitaro, Fujigaki Yoshihide, Yonemura Katsuhiko, Hishida Akira
First Department of Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Hamamatsu 431-3192, Japan.
Am J Med Sci. 2003 Aug;326(2):102-4. doi: 10.1097/00000441-200308000-00009.
A woman was admitted to the hospital with joint pain. She was also found to have pericardial effusion, renal dysfunction, pancytopenia, and positive antinuclear antibody; a diagnosis of systemic lupus erythematosus (SLE) was made. Although she had neither neurological symptoms nor fever, laboratory tests showed microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction. Therefore, we diagnosed her illness as SLE complicated by thrombotic microangiopathy (TMA). Plasmapheresis was performed in addition to immunosuppressive therapy. TMA improved rapidly and renal function improved gradually. The number of patients with SLE complicated by TMA is relatively small and the mortality rate is extremely high. A diagnosis of TMA is difficult to determine in patients with SLE because of the overlapping clinical symptoms. The data suggest that prompt induction of plasmapheresis in addition to immunosuppressive therapy is necessary in SLE patients having symptoms suspicious of TMA even before they fulfill the 5 symptoms typical of TMA.
一名女性因关节疼痛入院。还发现她有心包积液、肾功能不全、全血细胞减少以及抗核抗体阳性；诊断为系统性红斑狼疮（SLE）。尽管她既没有神经症状也没有发热，但实验室检查显示微血管病性溶血性贫血、血小板减少和肾功能不全。因此，我们将她的病情诊断为SLE合并血栓性微血管病（TMA）。除免疫抑制治疗外，还进行了血浆置换。TMA迅速改善，肾功能逐渐改善。SLE合并TMA的患者数量相对较少，死亡率极高。由于临床症状重叠，SLE患者很难诊断出TMA。数据表明，对于有TMA可疑症状的SLE患者，即使在出现TMA典型的5种症状之前，除免疫抑制治疗外，及时进行血浆置换诱导也是必要的。
