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系统性红斑狼疮患者局部肺内给予重组活化凝血因子 VII(rFVIIa)治疗弥漫性肺泡出血:一例报告

Treatment of diffuse alveolar hemorrhage in systemic lupus erythematosus patient with local pulmonary administration of factor VIIa (rFVIIa): a case report.

作者信息

Alabed Iehab B

机构信息

Internal Medicine Department, Al Hammadi Hospital, Riyadh, Saudi Arabia (IBA).

出版信息

Medicine (Baltimore). 2014 Sep;93(14):e72. doi: 10.1097/MD.0000000000000072.

Abstract

Diffuse alveolar hemorrhage (DAH) is a rare serious life-threatening complication in systemic lupus erythematosus (SLE) associated with a high mortality rate. The old standard treatment options include high-dose corticosteroids, cyclophosphamide, and plasmapheresis, which are unspecific, treating the underlying disease rather than the complication itself, and not effective. We report a case of DAH complicating SLE flare-up in a female patient treated with recombinant activated factor VII (rFVIIa) administered via the bronchoscope that showed clinical and radiological improvement. No toxicity or adverse events were observed with rFVII treatment. rFVII may be an effective treatment option for DAH in SLE patient.

摘要

弥漫性肺泡出血(DAH)是系统性红斑狼疮(SLE)中一种罕见但严重的危及生命的并发症,死亡率很高。以往的标准治疗方案包括大剂量皮质类固醇、环磷酰胺和血浆置换,这些方法缺乏特异性,针对的是基础疾病而非并发症本身,且效果不佳。我们报告了一例SLE病情发作合并DAH的女性患者,通过支气管镜给予重组活化因子VII(rFVIIa)治疗后,临床和影像学表现均有改善。rFVII治疗未观察到毒性或不良事件。rFVII可能是SLE患者DAH的一种有效治疗选择。

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