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Abstract：
To strengthen the understanding of rheumatic diseases (RDs) as the most common underlying conditions associated with acquired hemophilia (AH), a potentially fatal bleeding condition due to the development of autoantibodies or inhibitors to coagulation factor VIII, and rarely to factor IX, here we presented two cases of RDs associated AH to elucidate the disease progression, treatment, and prognosis. The presented 2 cases showed good responses to glucocorticoid (GC) and immunosuppressive agents. And then, a case-based systematic review was conducted to better understand the clinically practiced diagnosis and treatment of RDs associated AH. A total of 14 articles were included in the final literature review. All the identified 14 patients with underlying RDs and AH presented with bleeding symptoms, increased APTT, decreased FVIII activity, and positive FVIII inhibitors. Twelve of the 14 patients (85.7%) started an eradication of autoantibodies treatment with GC and immunosuppressive agents. Among which six patients achieved partial or complete remission, and four patients (28.6%) switched to Rituximab and responded well. Nine of the 14 patients received hemostasis therapy, including recombinant human FVIIa (rFVIIa). Two patients (14.3%) died due to mass bleeding and key organ failure. AH should be highly suspected in patients with RDs presenting spontaneous mucocutaneous or internal bleeding and an isolated prolonged APTT. Given the high morbidity of AH, it is important to facilitate efficient and proper management.
摘要：
为了加强对风湿性疾病（RD）作为与获得性血友病（AH）相关的最常见的潜在疾病的理解，由于自身抗体或凝血因子VIII抑制剂的发展，可能致命的出血状况，很少是因子IX，在这里，我们介绍了两个与AH相关的RD病例，以阐明疾病进展，治疗,和预后。2例患者对糖皮质激素(GC)和免疫抑制剂反应良好。然后,我们进行了一项以病例为基础的系统评价,以更好地了解RDS相关AH的临床实践诊断和治疗.共有14篇文章被纳入最终的文献综述。所有已确定的14名患有基础RD和AH的患者均出现出血症状，增加APTT,FVIII活性降低，和阳性FVIII抑制剂。14例患者中有12例（85.7％）开始用GC和免疫抑制剂根除自身抗体治疗。其中6例患者部分或完全缓解,4例患者(28.6%)改用利妥昔单抗,且反应良好.14例患者中有9例接受了止血治疗,包括重组人FVIIa(rFVIIa)。2例（14.3％）因大量出血和关键器官衰竭死亡。对于表现为自发性粘膜皮肤或内部出血以及孤立性延长的APTT的RD患者，应高度怀疑AH。鉴于AH的高发病率，重要的是促进有效和适当的管理。
