Lafferty T E, Smith J B, Schuster S J, DeHoratius R J
Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
Arthritis Rheum. 1997 Apr;40(4):775-8. doi: 10.1002/art.1780400426.
Acquired inhibitors of factor VIII have rarely been reported in the presence of systemic lupus erythematosus (SLE). Treatment is usually corticosteroids, with or without immunosuppressive agents such as cyclophosphamide or azathioprine. We report here case histories of 2 patients, one with documented SLE, the other with a forme fruste of SLE. Both patients had inadequate responses to corticosteroids and immunosuppressive agents. Both responded to intravenous immunoglobulin, with a decrease in their titers of factor VIII inhibitor and significantly decreased frequency of bleeding episodes despite persistent low-level inhibitor titers. Better control of their SLE symptoms and findings was also observed. Previously reported treatments of acquired factor VIII inhibitors in SLE are discussed.
获得性凝血因子 VIII 抑制剂在系统性红斑狼疮(SLE)患者中鲜有报道。治疗通常使用皮质类固醇,可联合或不联合免疫抑制剂如环磷酰胺或硫唑嘌呤。我们在此报告 2 例患者的病史,其中 1 例确诊为 SLE,另 1 例为 SLE 顿挫型。2 例患者对皮质类固醇和免疫抑制剂治疗反应不佳。二者均对静脉注射免疫球蛋白有反应,凝血因子 VIII 抑制剂滴度降低,出血发作频率显著下降,尽管抑制剂滴度仍维持在低水平。同时还观察到其 SLE 症状和体征得到了更好的控制。本文还讨论了先前报道的 SLE 患者获得性凝血因子 VIII 抑制剂的治疗方法。