系统性硬化症肾危象合并血栓性微血管病3例临床分析并文献复习
发布时间：2018-12-17 23:32
【摘要】：目的分析系统性硬化症肾危象(SRC)合并血栓性微血管病(TMA)的临床特点。方法收集2010年1月~2015年11月诊治的SRC患者4例,其中3例合并TMA,回顾性分析其临床资料,并对相关文献进行复习。结果 3例中女2例、男1例,年龄分别为59、67、76岁;SSc病程分别为1、2、13年,1例男性为弥漫型、2例女性为局限型,SRC罹患时间为2~4 d。3例均有急剧血压升高、肾功能不全、乳酸脱氢酶升高、血小板减少,外周血可见破碎红细胞,2例Coomb's试验阴性。3例均予以血管紧张素转换酶抑制剂,行连续肾脏替代疗法、静脉输注新鲜冰冻血浆、血浆置换各1例。治疗后病情均好转,1例1年后死亡。结论 SRC合并TMA并不少见,进展较快,病情凶险,应及早判断;治疗除应用血管紧张素转换酶抑制剂外,要进行肾脏替代治疗,必要时输注新鲜冰冻血浆。
[Abstract]:Objective to analyze the clinical features of renal crisis (SRC) complicated with thrombotic microangiopathy (TMA) in systemic sclerosis. Methods from January 2010 to November 2015, 4 patients with SRC were collected. The clinical data of 3 patients with TMA, were analyzed retrospectively, and the related literatures were reviewed. Results among the 3 cases, 2 cases were female, 1 case was male, the age was 59 years old and 67 years old, respectively. The course of SSc was 1 and 13 years, one male was diffuse type and two female were localized type. The onset time of SRC was 2d 4 in 3 cases, all had sharp blood pressure, renal insufficiency, lactate dehydrogenase and thrombocytopenia. The broken red blood cells were found in peripheral blood, 2 cases were negative in Coomb's test, 3 cases were treated with angiotensin converting enzyme inhibitor, followed by continuous renal replacement therapy, intravenous infusion of fresh frozen plasma, and plasma exchange in 1 case each. One case died one year later. Conclusion SRC combined with TMA is not uncommon, rapid progression and dangerous condition, should be judged early, in addition to the use of angiotensin converting enzyme inhibitors, renal replacement therapy, if necessary, infusion of fresh frozen plasma.
【作者单位】： 山东省千佛山医院;
【分类号】：R593.2
[Abstract]:Objective to analyze the clinical features of renal crisis (SRC) complicated with thrombotic microangiopathy (TMA) in systemic sclerosis. Methods from January 2010 to November 2015, 4 patients with SRC were collected. The clinical data of 3 patients with TMA, were analyzed retrospectively, and the related literatures were reviewed. Results among the 3 cases, 2 cases were female, 1 case was male, the age was 59 years old and 67 years old, respectively. The course of SSc was 1 and 13 years, one male was diffuse type and two female were localized type. The onset time of SRC was 2d 4 in 3 cases, all had sharp blood pressure, renal insufficiency, lactate dehydrogenase and thrombocytopenia. The broken red blood cells were found in peripheral blood, 2 cases were negative in Coomb's test, 3 cases were treated with angiotensin converting enzyme inhibitor, followed by continuous renal replacement therapy, intravenous infusion of fresh frozen plasma, and plasma exchange in 1 case each. One case died one year later. Conclusion SRC combined with TMA is not uncommon, rapid progression and dangerous condition, should be judged early, in addition to the use of angiotensin converting enzyme inhibitors, renal replacement therapy, if necessary, infusion of fresh frozen plasma.
【作者单位】： 山东省千佛山医院;
【分类号】：R593.2
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1 高玉祥;一例硬皮病发生肾危象伴Vogt-koyanagi-Harada综合征[J];国外医学.皮肤病学分册;1986年05期
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