Department of Medicine (Division of Clinical Immunology and Rheumatology), School of Medicine, The University of Alabama at Birmingham, Birmingham, Alabama, USA.
Lupus. 2010 Oct;19(11):1331-6. doi: 10.1177/0961203310375264. Epub 2010 Aug 9.
The aim of this study was to characterize the clinical features of familial lupus, and determine its influence on damage accrual and survival using data from LUMINA, a longitudinal multiethnic US cohort. Familial lupus was defined as patients with a first-degree relative with systemic lupus erythematosus. Relative risks were estimated by logistic regression; odds ratios (ORs) and their 95% confidence intervals (CIs) were the measure of association for familial lupus. Hazard ratios were calculated using Cox proportional hazards adjusted for potential confounders for damage and survival. Of 644 patients, 32 had familial and 612 had sporadic lupus; both groups were of comparable age (~36 years). Patients with familial lupus were, in decreasing order of frequency, siblings, parents and children. In multivariable analyses, mucosal ulcers (OR = 1.92, 95% CI 0.65-5.70), mitral valve prolapse (OR = 1.74, 95% CI 0.50-6.10), cerebrovascular disease (OR = 4.18, 95% CI 0.98-17.76) and oral contraceptive use (ever/never; OR = 2.51, 95% CI 0.88-7.19) were more likely in familial lupus, but a history of low platelet count (<150,000/mm(3); OR=0.31, 95% CI 0.08-1.17) and pulmonary disease activity (OR=0.39, 95% CI 0.14-1.20) were less likely. However, none of these associations reached statistical significance. Familial lupus was not significantly associated with a shorter time to either damage accrual or death (HR = 0.77, 95% CI 0.37-1.59, p = 0.4746 and HR = 0.20, 95% CI 0.03-1.47, p = 0.2020, respectively). We conclude that although some clinical differences were observed between patients with familial and sporadic lupus, familial lupus was not associated with a significantly greater disease burden (damage, survival) than sporadic lupus.
本研究旨在描述家族性狼疮的临床特征，并利用 LUMINA 纵向多民族美国队列的数据来确定其对累积损害和生存的影响。家族性狼疮定义为一级亲属患有系统性红斑狼疮的患者。采用逻辑回归估计相对风险；比值比 (OR) 及其 95%置信区间 (CI) 用于家族性狼疮的关联度量。使用 Cox 比例风险模型调整潜在混杂因素后，计算损伤和生存的风险比。在 644 例患者中，32 例为家族性狼疮，612 例为散发性狼疮；两组的年龄（~36 岁）相近。家族性狼疮患者依次为兄弟姐妹、父母和子女。在多变量分析中，黏膜溃疡（OR=1.92，95%CI 0.65-5.70）、二尖瓣脱垂（OR=1.74，95%CI 0.50-6.10）、脑血管疾病（OR=4.18，95%CI 0.98-17.76）和口服避孕药使用（曾用/从未使用；OR=2.51，95%CI 0.88-7.19）在家族性狼疮中更为常见，但血小板计数低（<150,000/mm(3)；OR=0.31，95%CI 0.08-1.17）和肺部疾病活动（OR=0.39，95%CI 0.14-1.20）较少见。然而，这些关联均无统计学意义。家族性狼疮与累积损害或死亡的时间均无显著相关性（HR=0.77，95%CI 0.37-1.59，p=0.4746 和 HR=0.20，95%CI 0.03-1.47，p=0.2020）。综上，尽管在家族性狼疮和散发性狼疮患者中观察到了一些临床差异，但家族性狼疮与疾病负担（损伤、生存）无显著相关性，并不比散发性狼疮更严重。
