Jeffries M, Hamadeh F, Aberle T, Glenn S, Kamen D L, Kelly J A, Reichlin M, Harley J B, Sawalha A H
College of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA.
Lupus. 2008 Aug;17(8):739-43. doi: 10.1177/0961203308090990.
Systemic lupus erythematosus is a chronic autoimmune disease that can be associated with a variety of haematological manifestations. We identified 76 patients with haemolytic anaemia in a cohort of 1251 unrelated female lupus patients enrolled in our studies. The presence of the various American College of Rheumatology clinical criteria for lupus and serological specificities were determined in lupus patients with haemolytic anaemia and compared with a group of race-matched control lupus patients without haemolytic anaemia. Clinical data were obtained from medical records, and serological specificities were determined in our clinical immunology laboratory at OMRF. The presence of haemolytic anaemia in lupus patients was associated with a higher frequency of proteinuria (OR = 2.70, P = 0.000031), urinary cellular casts (OR = 2.83, P = 0.000062), seizures (OR = 2.96, P = 0.00024), pericarditis (OR = 2.21, P = 0.0019), pleuritis (OR = 1.72, P = 0.028) and lymphopenia (OR = 1.79, P = 0.015). These findings were independent of the presence of thrombocytopenia, which was approximately five times more common in lupus patients with haemolytic anaemia. Lupus patients with haemolytic anaemia were about 8 years younger than lupus patients without haemolytic anaemia at the time of disease onset (P = 0.000001). In the absence of thrombocytopenia, lupus patients with haemolytic anaemia were approximately two times more likely to have anti-dsDNA antibodies (P = 0.024). The presence of haemolytic anaemia is associated with a subset of lupus characterized by a younger age of disease onset, and a more severe disease with a higher likelihood of renal involvement, seizures, serositis and other cytopenias.
系统性红斑狼疮是一种慢性自身免疫性疾病，可伴有多种血液学表现。在参与我们研究的1251名无亲缘关系的女性狼疮患者队列中，我们识别出76例溶血性贫血患者。在患有溶血性贫血的狼疮患者中确定了各种美国风湿病学会狼疮临床标准的存在情况以及血清学特异性，并与一组种族匹配的无溶血性贫血的对照狼疮患者进行比较。临床数据从病历中获取，血清学特异性在OMRF的临床免疫实验室中测定。狼疮患者中溶血性贫血的存在与蛋白尿（OR = 2.70，P = 0.000031）、尿细胞管型（OR = 2.83，P = 0.000062）、癫痫发作（OR = 2.96，P = 0.00024）、心包炎（OR = 2.21，P = 0.0019）、胸膜炎（OR = 1.72，P = 0.028）和淋巴细胞减少（OR = 1.79，P = 0.015）的较高发生率相关。这些发现独立于血小板减少症的存在，血小板减少症在患有溶血性贫血的狼疮患者中大约常见五倍。在疾病发作时，患有溶血性贫血的狼疮患者比无溶血性贫血的狼疮患者年轻约8岁（P = 0.000001）。在无血小板减少症的情况下，患有溶血性贫血的狼疮患者出现抗双链DNA抗体的可能性大约高两倍（P = 0.024）。溶血性贫血的存在与一部分狼疮相关，其特征为疾病发病年龄较轻，疾病更严重，肾脏受累、癫痫发作、浆膜炎和其他血细胞减少症的可能性更高。
