Tang Qi, Liao Jiafen, Xie Xi
Department of Rheumatology and Immunology, the Second Xiangya Hospital, Central South University, Changsha, People's Republic of China.
J Inflamm Res. 2022 Aug 3;15:4385-4393. doi: 10.2147/JIR.S369288. eCollection 2022.
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.
为加强对风湿性疾病（RDs）作为与获得性血友病（AH）相关的最常见基础疾病的理解，AH是一种由于自身抗体或凝血因子VIII抑制剂（很少是因子IX）的产生而导致的潜在致命性出血疾病，在此我们报告两例与RDs相关的AH病例，以阐明疾病进展、治疗及预后情况。所报告的2例病例对糖皮质激素（GC）和免疫抑制剂表现出良好反应。然后，进行了一项基于病例的系统综述，以更好地了解与RDs相关的AH的临床实际诊断和治疗。最终的文献综述共纳入14篇文章。所有14例确诊的患有基础RDs和AH的患者均出现出血症状、活化部分凝血活酶时间（APTT）延长、因子VIII（FVIII）活性降低以及FVIII抑制剂阳性。14例患者中有12例（85.7%）开始采用GC和免疫抑制剂进行清除自身抗体治疗。其中6例患者实现部分或完全缓解，4例患者（28.6%）改用利妥昔单抗并反应良好。14例患者中有9例接受了止血治疗，包括重组人凝血因子VIIa（rFVIIa）。2例患者（14.3%）因大量出血和关键器官衰竭死亡。对于出现自发性黏膜皮肤或内出血且APTT单独延长的RDs患者，应高度怀疑AH。鉴于AH的高发病率，促进高效且恰当的管理很重要。
