Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus: Experiences From a Chinese Tertiary Medical Center.
Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus: Experiences From a Chinese Tertiary Medical Center.
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继发于系统性红斑狼疮的因子 VIII 和冯维勒布兰德因子获得性抑制剂的特点：来自中国三级医疗中心的经验。
DOI：
10.1097/rhu.0000000000001284
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发表时间：
2021-08-01
期刊：
影响因子：
--
通讯作者：
Zhu LX
中科院分区：
文献类型：
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作者：
Cao XY;Li MT;Zhang X;Zhao Y;Zeng XF;Zhang FC;Hou Y;Zhu LX
由于获得性血友病（AH）是一种罕见的实体系统性红斑狼疮（SLE），我们的目的是探讨SLE相关的AH在中国患者的临床特征。这是一项1986年至2018年在中国一家大型三级医院进行的病历审查研究。我们使用国际疾病分类检索了北京协和医院的病例数据库。收集SLE相关AH患者的临床资料。共有9282例SLE患者住院。确定了6例女性SLE相关AH患者。4例患者患有获得性血友病A（AHA），2例患者患有获得性von Willebrand综合征。平均年龄33.67 ± 13.77岁。5例患者有活动性疾病。在诊断AH时测量的平均SLE疾病活动指数为10.50 ± 5.28。活化部分凝血活酶时间的平均水平为86.5秒。1例继发性抗磷脂综合征合并AHA，3年后发生肺栓塞。经免疫抑制剂治疗和对症治疗后，总缓解率为83.3%。SLE相关AH发生率低。SLE患者AH的发生常与疾病活动有关。AH可能是SLE的首发临床表现。继发性抗磷脂综合征和AHA可出现在同一SLE患者中。早期和积极的治疗有助于良好的预后。
Because acquired hemophilia (AH) is a rare entity in systemic lupus erythematosus (SLE), we aimed to investigate the clinical features of SLE-related AH in Chinese patients. This is a medical records review study carried out at a large tertiary care hospital in China from years 1986 to 2018. We searched the case database in Peking Union Medical College Hospital using the International Classification of Diseases. The clinical data on SLE-related AH patients were collected. A total of 9282 SLE patients had been hospitalized. Six female SLE-related AH patients were identified. Four patients had acquired hemophilia A (AHA), and 2 patients had acquired von Willebrand syndrome. Their mean age was 33.67 ± 13.77 years. Five patients had active disease. The mean SLE disease activity index measured at the time of diagnosis of AH was 10.50 ± 5.28. The average level of activated partial thromboplastin time was 86.5 seconds. Coexistence of secondary antiphospholipid syndrome and AHA was found in one case, and pulmonary embolism was observed 3 years later. After immunosuppressive therapy and symptomatic treatment, an overall remission rate of 83.3% was achieved. The frequency of SLE-related AH was low. The development of AH in SLE patients frequently occurs with active disease. The AH could be the first clinical presentation of SLE. Secondary antiphospholipid syndrome and AHA could appear in the same SLE patient. Early and aggressive treatment contributes to a favorable prognosis.