巴西原发性抗磷脂综合征患者与非巴西原发性抗磷脂综合征患者在抗磷脂综合征临床试验和国际网络联盟（APS ACTION）临床数据库和存储库中的临床和实验室特征。
Clinical and laboratory characteristics of Brazilian versus non-Brazilian primary antiphospholipid syndrome patients in AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) clinical database and repository.
机构信息
University of São Paulo, Av. Dr. Arnaldo 455, Third Floor, Room 3109, São Paulo, 01246903, Brazil.
Federal University of Juiz de Fora, Minas Gerais, Brazil.
出版信息
Adv Rheumatol. 2021 Oct 28;61(1):64. doi: 10.1186/s42358-021-00222-3.
BACKGROUND
Antiphospholipid syndrome (APS) is characterized by episodes of thrombosis, obstetric morbidity or both, associated with persistently positive antiphospholipid antibodies (aPL). Studying the profile of a rare disease in an admixed population is important as it can provide new insights for understanding an autoimmune disease. In this sense of miscegenation, Brazil is characterized by one of the most heterogeneous populations in the world, which is the result of five centuries of interethnic crosses of people from three continents. The objective of this study was to compare the clinical and laboratory characteristics of Brazilian vs. non-Brazilian primary antiphospholipid syndrome (PAPS) patients.
METHODS
We classified PAPS patients into 2 groups: Brazilian PAPS patients (BPAPS) and PAPS patients from other countries (non-BPAPS). They were compared regarding demographic characteristics, criteria and non-criteria APS manifestations, antiphospholipid antibody (aPL) profile, and the adjusted Global Antiphospholipid Syndrome Score (aGAPSS).
RESULTS
We included 415 PAPS patients (88 [21%] BPAPS and 327 [79%] non-BPAPS). Brazilian patients were significantly younger, more frequently female, sedentary, obese, non-white, and had a higher frequency of livedo (25% vs. 10%, p < 0.001), cognitive dysfunction (21% vs. 8%, p = 0.001) and seizures (16% vs. 7%, p = 0.007), and a lower frequency of thrombocytopenia (9% vs. 18%, p = 0.037). Additionally, they were more frequently positive for lupus anticoagulant (87.5% vs. 74.6%, p = 0.01), and less frequently positive to anticardiolipin (46.6% vs. 73.7%, p < 0.001) and anti-ß2-glycoprotein-I (13.6% vs. 62.7%, p < 0.001) antibodies. Triple aPL positivity was also less frequent (8% vs. 41.6%, p < 0.001) in Brazilian patients. Median aGAPSS was lower in the Brazilian group (8 vs. 10, p < 0.0001). In the multivariate analysis, BPAPS patients still presented more frequently with livedo, cognitive dysfunction and sedentary lifestyle, and less frequently with thrombocytopenia and triple positivity to aPL. They were also less often white.
CONCLUSIONS
Our study suggests a specific profile of PAPS in Brazil with higher frequency of selected non-criteria manifestations and lupus anticoagulant positivity. Lupus anticoagulant (not triple positivity) was the major aPL predictor of a classification criteria event.
背景
抗磷脂综合征（APS）的特征是血栓形成、产科发病率或两者兼有，并与持续存在的抗磷脂抗体（aPL）有关。在混合人群中研究罕见疾病的特征很重要，因为这可以为理解自身免疫性疾病提供新的见解。在这种混合的情况下，巴西是世界上人口最多样化的国家之一，这是三个大陆的人种五个世纪杂交的结果。本研究的目的是比较巴西与非巴西原发性抗磷脂综合征（PAPS）患者的临床和实验室特征。
方法
我们将 PAPS 患者分为 2 组：巴西 PAPS 患者（BPAPS）和来自其他国家的 PAPS 患者（非 BPAPS）。比较两组患者的人口统计学特征、APS 标准和非标准表现、抗磷脂抗体（aPL）谱以及调整后的全球抗磷脂综合征评分（aGAPSS）。
结果
我们纳入了 415 名 PAPS 患者（88 名[21%]为 BPAPS，327 名[79%]为非 BPAPS）。巴西患者明显更年轻、更多为女性、久坐、肥胖、非白人，且更常出现皮肤网状青斑（25%比 10%，p<0.001）、认知功能障碍（21%比 8%，p=0.001）和癫痫发作（16%比 7%，p=0.007），血小板减少症（9%比 18%，p=0.037）发生率较低。此外，巴西患者狼疮抗凝物（87.5%比 74.6%，p=0.01）阳性率较高，抗心磷脂抗体（46.6%比 73.7%，p<0.001）和抗β2-糖蛋白 I 抗体（13.6%比 62.7%，p<0.001）阳性率较低。三阳性 aPL 也较少见（8%比 41.6%，p<0.001）。巴西组的中位 aGAPSS 较低（8 比 10，p<0.0001）。在多变量分析中，BPAPS 患者仍更常出现皮肤网状青斑、认知功能障碍和久坐的生活方式，血小板减少症和三阳性 aPL 的发生率较低。他们也较少是白人。
结论
我们的研究表明，巴西的 PAPS 具有特定的特征，表现为更频繁的特定非标准表现和狼疮抗凝物阳性。狼疮抗凝物（而非三阳性）是分类标准事件的主要 aPL 预测因素。
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