Seaman D E, Londino A V, Kwoh C K, Medsger T A, Manzi S
Department of Medicine, Graduate School of Public Health, University of Pittsburgh School of Medicine, PA 15213, USA.
Pediatrics. 1995 Dec;96(6):1040-5.
Antiphospholipid antibodies (aPLs) have been extensively studied in adults with systemic lupus erythematosus (SLE) and have been associated with arterial and venous thrombosis, thrombocytopenia, neurologic disorders, and recurrent fetal loss. In contrast, very little is known about the frequency and clinical significance of aPLs in pediatric SLE. This study was designed to determine the frequency of aPLs in pediatric SLE and the temporally associated clinical manifestations.
We studied 29 consecutive patients with onset of SLE in childhood seen in the Pediatric Rheumatology Clinic at the University of Pittsburgh, Children's Hospital, between 1985 and 1992. We defined aPL as the presence of a lupus anticoagulant (LAC), immunoglobulin G or immunoglobulin M anticardiolipin antibodies (aCLs), or a biologic false-positive serologic test for syphilis determined by a VDRL test. Clinical manifestations were temporally correlated to the presence of aPLs if they occurred within 6 months.
Overall, 19 (65%) of 29 children with SLE had one of the three laboratory abnormalities defining aPL. LAC was detected in 16 (62%) of 26, aCL in 18 (66%) of 27, and false-positive VDRL test results in 11 (39%) of 28. Twenty-five of the 29 patients had all three tests performed. In 10 patients, all three tests were abnormal. The presence of thrombosis in 7 patients (4 venous, 2 arterial, and 1 both) was associated with a positive aPL, specifically aCL. The presence of an aPL was significantly associated with anti-double-stranded DNA antibodies, but not with neuropsychiatric manifestations or with thrombocytopenia. The presence of an aCL was significantly associated with hemolytic anemia. A prolonged prothrombin time, in the setting of an LAC (all with a prolonged activated partial thromboplastin time), was associated with life-threatening disease in 6 of 15 patients.
Sixty-five percent of 29 consecutive pediatric patients with SLE had evidence of aPL. The presence of aPL, specifically aCL, was significantly associated with thrombotic events. The presence of a prolonged prothrombin time in the setting of an LAC may be a marker of more serious disease in pediatric SLE.
抗磷脂抗体（aPLs）在成年系统性红斑狼疮（SLE）患者中已得到广泛研究，与动脉和静脉血栓形成、血小板减少、神经系统疾病及反复流产相关。相比之下，对于儿童SLE中aPLs的发生率及临床意义却知之甚少。本研究旨在确定儿童SLE中aPLs的发生率及其相关的临床表现。
我们研究了1985年至1992年间在匹兹堡大学儿童医院儿科风湿病诊所就诊的29例儿童期起病的SLE患者。我们将aPL定义为存在狼疮抗凝物（LAC）、免疫球蛋白G或免疫球蛋白M抗心磷脂抗体（aCLs），或通过性病研究实验室（VDRL）试验确定的梅毒生物学假阳性血清学试验。如果临床表现发生在6个月内，则与aPLs的存在进行时间相关性分析。
总体而言，29例SLE患儿中有19例（65%）存在定义aPL的三项实验室异常之一。26例中有16例（62%）检测到LAC，27例中有18例（66%）检测到aCL，28例中有11例（39%）VDRL试验结果为假阳性。29例患者中有25例进行了所有三项检测。10例患者三项检测均异常。7例患者（4例静脉血栓、2例动脉血栓和1例动静脉血栓）出现血栓与aPL阳性相关，特别是aCL。aPL的存在与抗双链DNA抗体显著相关，但与神经精神表现或血小板减少无关。aCL的存在与溶血性贫血显著相关。在LAC存在的情况下（所有患者活化部分凝血活酶时间均延长），凝血酶原时间延长与15例患者中的6例危及生命的疾病相关。
29例连续的儿童SLE患者中有65%有aPL证据。aPL特别是aCL的存在与血栓形成事件显著相关。在LAC存在的情况下凝血酶原时间延长可能是儿童SLE中更严重疾病的一个标志。
