Bortoli Rodrigo, Monticielo Odirlei A, Chakr Rafael M, Palominos Penélope E, Rohsig Liane M, Kohem Charles L, Xavier Ricardo M, Brenol João Carlos T
Department of Internal Medicine, Division of Rheumatology, Hospital de Clinicas de Porto Alegre, Cep 90035-903, Porto Alegre, Rio Grande do Sul, Brazil.
Semin Arthritis Rheum. 2009 Aug;39(1):61-5. doi: 10.1016/j.semarthrit.2008.03.005. Epub 2008 Jun 2.
Rare patients with systemic lupus erythematosus (SLE) patients exhibit anticoagulants that interfere in the earlier stages of the intrinsic coagulation pathway, such as those involving factor XI (FXI). The objectives of our study were to describe the presence of an acquired inhibitor to FXI causing a life-threatening bleeding disorder in an SLE patient and to review the association of this coagulopathy with SLE.
We describe the clinical presentation of an SLE patient with an acquired FXI inhibitor. We reviewed the scientific literature using the MEDLINE database searching the following combinations of terms: "SLE and Factor XI," "SLE and Factor XI inhibitor," and "Factor XI inhibitor," from 1964 to 2007.
A 20-year-old woman with a 6-year history of SLE was admitted to the hospital because of severe life-threatening abdominal bleeding due to a ruptured ovarian cyst. This hemorrhagic event was related to the presence of an FXI inhibitor. We reviewed another 13 SLE patients with this condition, 8 of whom had bleeding events. Most patients had manifestations of active SLE, and prednisone was used as the primary treatment.
SLE activity seems to be associated with the production of antibodies directed against FXI, which may cause important coagulopathies, especially bleeding events. The inhibitor disappeared after immunosuppressive therapy for SLE in most cases, suggesting that the appearance of this inhibitor is immune mediated. Although the majority of cases with the FXI inhibitor are not fatal, it should be suspected and investigated in SLE patients, especially those with abnormal clotting tests.
罕见的系统性红斑狼疮（SLE）患者会出现干扰内源性凝血途径早期阶段的抗凝物质，例如涉及因子XI（FXI）的抗凝物质。我们研究的目的是描述一名SLE患者出现获得性FXI抑制剂导致危及生命的出血性疾病的情况，并回顾这种凝血病与SLE的关联。
我们描述了一名患有获得性FXI抑制剂的SLE患者的临床表现。我们使用MEDLINE数据库检索科学文献，搜索了以下术语组合：“SLE与因子XI”、“SLE与因子XI抑制剂”以及“因子XI抑制剂”，时间跨度为1964年至2007年。
一名有6年SLE病史的20岁女性因卵巢囊肿破裂导致严重危及生命的腹部出血而入院。这一出血事件与FXI抑制剂的存在有关。我们回顾了另外13例患有这种疾病的SLE患者，其中8例有出血事件。大多数患者有活动性SLE的表现，泼尼松被用作主要治疗药物。
SLE活动似乎与针对FXI的抗体产生有关，这可能导致重要的凝血病，尤其是出血事件。在大多数情况下，免疫抑制治疗SLE后抑制剂消失，这表明这种抑制剂的出现是免疫介导的。尽管大多数有FXI抑制剂的病例并非致命，但在SLE患者中，尤其是凝血试验异常的患者中，应怀疑并进行调查。
