Kokori S I, Ioannidis J P, Voulgarelis M, Tzioufas A G, Moutsopoulos H M
Department of Pathophysiology, University of Athens School of Medicine, Greece.
Am J Med. 2000 Feb 15;108(3):198-204. doi: 10.1016/s0002-9343(99)00413-1.
We sought to evaluate the clinical and serologic associations with, and outcomes of, autoimmune hemolytic anemia, as compared with other types of anemia, in patients with systemic lupus erythematosus (SLE).
We studied 41 consecutive patients with SLE with clinically manifest autoimmune hemolytic anemia, including 27 (66%) in whom hemolysis was the initial disease manifestation. We matched each patient for age and disease duration with a patient with SLE with anemia resulting from a different cause.
The 41 patients had a total of 50 episodes of autoimmune hemolytic anemia. The recurrence rate was 4 per 100 person-years. Cases and controls had similar mean (+/- SD) lupus activity indexes (2.1 +/- 1.5 vs 2.4 +/- 1.3, P = 0.5). Patients with autoimmune hemolytic anemia at any time could be distinguished from patients with other causes of anemia, because they were more likely to have elevated titers of IgG anticardiolipin antibodies [odds ratio (OR) = 5.8; 95% confidence interval (CI), 1.4 to 24] and thrombosis (OR = 4.6; 95% CI, 1.0 to 21). Autoimmune hemolytic anemia at the onset of SLE was independently associated with renal involvement (OR = 5.4; 95% CI, 1.0 to 28), thrombocytopenia (OR = 7.3; 95% CI, 1.1 to 48), and possibly thrombotic episodes during follow-up (OR = 11; 95% CI, 0.8 to 160) when compared with controls with other types of anemia at the onset of SLE.
Autoimmune hemolytic anemia usually occurs at the onset of SLE, and its recurrence rate is low among treated patients. The association with IgG anticardiolipin antibodies and thrombosis suggests that the occurrence of autoimmune hemolytic anemia may define a subgroup of patients with SLE who have characteristic serologic and clinical manifestations.
我们试图评估系统性红斑狼疮（SLE）患者中自身免疫性溶血性贫血与其他类型贫血相比的临床和血清学关联及结局。
我们研究了41例连续的有临床表现的自身免疫性溶血性贫血的SLE患者，其中27例（66%）溶血是最初的疾病表现。我们将每位患者与年龄和病程匹配的因不同原因导致贫血的SLE患者进行配对。
41例患者共发生50次自身免疫性溶血性贫血发作。复发率为每100人年4次。病例组和对照组的狼疮活动指数均值（±标准差）相似（2.1±1.5对2.4±1.3，P = 0.5）。任何时候患有自身免疫性溶血性贫血的患者都可与其他贫血原因的患者区分开来，因为他们更可能有IgG抗心磷脂抗体滴度升高[比值比（OR）= 5.8；95%置信区间（CI），1.4至24]和血栓形成（OR = 4.6；95% CI，1.0至21）。与SLE发病时其他类型贫血的对照组相比，SLE发病时的自身免疫性溶血性贫血与肾脏受累（OR = 5.4；95% CI，1.0至28）、血小板减少（OR = 7.3；95% CI，1.1至48）以及随访期间可能的血栓形成事件（OR = 11；95% CI，0.8至160）独立相关。
自身免疫性溶血性贫血通常发生在SLE发病时，且在接受治疗的患者中复发率较低。与IgG抗心磷脂抗体和血栓形成的关联表明，自身免疫性溶血性贫血的发生可能定义了一组具有特征性血清学和临床表现的SLE患者亚组。
