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系统性红斑狼疮中的抗磷脂抗体与抗磷脂综合征。对500例连续患者的前瞻性分析。

Antiphospholipid antibodies and the antiphospholipid syndrome in systemic lupus erythematosus. A prospective analysis of 500 consecutive patients.

作者信息

Alarcón-Segovia D, Delezé M, Oria C V, Sánchez-Guerrero J, Gómez-Pacheco L, Cabiedes J, Fernández L, Ponce de León S

机构信息

Department of Immunology, Instituto Nacional de la Nutrición Salvador Zubirán, Mexico City, Mexico.

出版信息

Medicine (Baltimore). 1989 Nov;68(6):353-65. doi: 10.1097/00005792-198911000-00003.

DOI:10.1097/00005792-198911000-00003
PMID:2811661
Abstract

Five hundred consecutive patients with systemic lupus erythematosus (SLE) were entered into a prospective study of anticardiolipin antibodies (ACLA) in their 3 major immunoglobulin isotypes and followed thereafter with repeated testing for a mean period of nearly 8 months. Manifestations of SLE that were strongly associated with ACLA included venous thrombosis (particularly when recurrent), thrombocytopenia, hemolytic anemia, recurrent fetal loss, and leg ulcers. Other manifestations found to be associated with ACLA were arterial occlusions, transverse myelitis, and pulmonary hypertension. Conversely, we found no relationship between ACLA and migraine, convulsions, transient ischemic attacks, psychoses, or avascular necrosis of bone. No relationship was found between the presence of ACLA and that of anti-DNA antibodies studied in the same serum sample. Association with ACLA grew stronger and titers became higher in patients with several of the associated manifestations. Statistical analyses revealed the existence of a syndrome, the antiphospholipid syndrome, comprising 2 or more manifestations in conjunction with ACLA titers 5 standard deviations above the mean of normal control subjects, particularly if ACLA had been positive on at least 2 occasions. We propose that such criteria could be applied to the definition of the antiphospholipid syndrome. The presence and the titers of these antibodies related to disease activity and titer decreased by treatment, particularly when they were of the IgM isotype. Patients in whom a thrombotic episode occurred during the course of the study were observed to have a coincident decrease in ACLA titers, a finding that might indicate consumption of the antibody during the event. Treatment and the resulting inactivation of disease appear to have independent effects on ACLA titers. Physicians should therefore be cautious in prescribing high doses of corticosteroids or immunosuppressants to patients with SLE solely because they have high titers of ACLA.

摘要

500例连续性系统性红斑狼疮(SLE)患者进入一项关于抗心磷脂抗体(ACLA)3种主要免疫球蛋白亚型的前瞻性研究,此后进行重复检测,平均随访近8个月。与ACLA密切相关的SLE表现包括静脉血栓形成(尤其是复发时)、血小板减少、溶血性贫血、反复流产和腿部溃疡。发现与ACLA相关的其他表现有动脉闭塞、横贯性脊髓炎和肺动脉高压。相反,我们发现ACLA与偏头痛、惊厥、短暂性脑缺血发作、精神病或骨缺血性坏死之间无关联。在同一血清样本中研究发现,ACLA的存在与抗DNA抗体的存在之间无关联。在有几种相关表现的患者中,与ACLA的关联更强且滴度更高。统计分析揭示了一种综合征即抗磷脂综合征的存在,该综合征由2种或更多种表现以及ACLA滴度高于正常对照受试者平均值5个标准差组成,特别是如果ACLA至少2次呈阳性。我们建议这些标准可应用于抗磷脂综合征的定义。这些抗体的存在和滴度与疾病活动相关,且通过治疗滴度下降,尤其是当它们为IgM亚型时。在研究过程中发生血栓形成事件的患者,观察到其ACLA滴度同时下降,这一发现可能表明在该事件中抗体被消耗。治疗及由此导致的疾病失活似乎对ACLA滴度有独立影响。因此,医生在仅因SLE患者ACLA滴度高就开具高剂量皮质类固醇或免疫抑制剂时应谨慎。

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