我们介绍了一例20多岁的女性,其系统性红斑狼疮(SLE)治疗不足。她出现了大量月经出血,随着鼻和牙龈出血恶化超过3个月。她的家族没有出血史,童年,牙科手术或分娩。评估排除了结构性原因,揭示活化的部分凝血活酶时间延长(混合研究的不完全校正),正常凝血酶原时间,中度血小板减少症,狼疮抗凝和抗磷脂酰丝氨酸/凝血酶原抗体阳性两次,相隔12周。进一步评估显示vonWillebrand因子(vWF)水平非常低(<5%)。她用脉冲甲基强的松龙治疗了3天,导致症状完全缓解,vWF水平提高到130%。没有出血史,家族史,在伴有抗磷脂抗体阳性的SLE患者中,vWF极低的存在及其对皮质类固醇的反应导致获得性vWF综合征被诊断为粘膜出血的原因.她服用羟氯喹后出院,霉酚酸酯和逐渐减少口服皮质类固醇。
We present a case of a woman in her 20s with inadequately treated systemic lupus erythematosus (SLE). She presented with heavy menstrual bleeding, along with nasal and gum bleeding worsening over 3 months. There was no bleeding history in her family, childhood, dental procedures or childbirth. Evaluation ruled out structural causes, revealing prolonged activated partial thromboplastin time (incomplete correction on mixing studies), normal prothrombin time, moderate thrombocytopenia, and lupus anticoagulant and anti-phosphatidylserine/prothrombin antibody positivity twice, 12 weeks apart. Further evaluation showed very low von Willebrand factor (vWF) levels (<5%). She was treated with pulse methylprednisolone for 3 days, resulting in complete symptom resolution and improvement in vWF levels to 130%. The absence of bleeding history, family history, presence of very low vWF and its response to corticosteroids led to a diagnosis of acquired vWF syndrome as the cause of mucosal bleeding in an SLE patient with concomitant positive antiphospholipid antibody. She was discharged on hydroxychloroquine, mycophenolate mofetil and tapering oral corticosteroids.