Alarcón-Segovia D, Pérez-Vázquez M E, Villa A R, Drenkard C, Cabiedes J
Department of Immunology and Rheumatology, Instituto Nacional de la Nutrición Salvador Zubirán, Mexico City, Mexico.
Semin Arthritis Rheum. 1992 Apr;21(5):275-86. doi: 10.1016/0049-0172(92)90021-5.
Ten percent of 667 consecutive systemic lupus erythematosus (SLE) patients were considered to have definite antiphospholipid syndrome (aPLS) because they had two or more antiphospholipid (aPL)-related clinical manifestations and aPL titers more than 5 SD above the mean of normal controls. Another 14% had either one aPL-related manifestation but high titers of the antibody or two manifestations and low aPL titers (probable aPLS). One fourth of the patients had no manifestations but high titers, one manifestation and low titers, or two or more manifestations and negative aPL titers ("doubtful" aPLS); the other half were considered negative for aPLS. In patients with high-titer aPL, the number of aPL-related manifestations was influenced by disease duration and number of pregnancies, indicating potential mobility of category with time or with risk of recurrent pregnancy loss. Patients with two or more manifestations but variable aPL levels differed in immunosuppressive treatment and in the number of times they had been tested, indicating potential mobility of category with lower treatment and/or further aPL testing. Patients with definite aPLS had increased risk of cutaneous vasculitis, peripheral neuropathy, seizures, psychosis, transient ischemic attacks, and leukopenia. In 11 of 52 SLE patients with definite aPLS the initial manifestation was related to aPL, and in 16 it concurred with an unrelated one. Only two patients fulfilled criteria for aPLS before having other evidence of SLE. The authors conclude that aPLS occurring within SLE is part of the disease rather than an associated condition and propose the use of definite and probable classification categories. These criteria, with appropriate follow-up and clinical and serological exclusion clauses for potential primary conditions, could also be applied to primary aPLS.
在连续的667例系统性红斑狼疮（SLE）患者中，10%被认为患有明确的抗磷脂综合征（aPLS），因为他们有两种或更多与抗磷脂（aPL）相关的临床表现，且aPL滴度比正常对照均值高出5个标准差以上。另外14%的患者有1种与aPL相关的表现但抗体滴度高，或者有2种表现但aPL滴度低（可能为aPLS）。四分之一的患者没有临床表现但aPL滴度高，有1种表现且滴度低，或者有2种或更多表现但aPL滴度为阴性（“可疑”aPLS）；另一半被认为aPLS为阴性。在aPL滴度高的患者中，与aPL相关的表现数量受疾病持续时间和妊娠次数的影响，表明随着时间推移或反复流产风险，分类可能会发生变化。有2种或更多表现但aPL水平不同的患者在免疫抑制治疗及检测次数方面存在差异，表明随着治疗减少和/或进一步进行aPL检测，分类可能会发生变化。明确患有aPLS的患者发生皮肤血管炎、周围神经病变、癫痫、精神病、短暂性脑缺血发作和白细胞减少的风险增加。在52例明确患有aPLS的SLE患者中，11例最初的表现与aPL有关，16例则与不相关的表现同时出现。只有2例患者在出现SLE的其他证据之前就符合aPLS的标准。作者得出结论，SLE中出现的aPLS是疾病的一部分而非相关病症，并建议使用明确和可能的分类类别。这些标准，加上对潜在原发性疾病进行适当随访以及临床和血清学排除条款，也可应用于原发性aPLS。
