Rheumatology Division - Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo (FMUSP), Sao Paulo, Brazil.
Lupus. 2020 Mar;29(3):256-262. doi: 10.1177/0961203320901598. Epub 2020 Jan 27.
Antiphospholipid syndrome (APS) is an acquired thrombophilia that affects young productive individuals, with permanent damage and negative impact on quality of life. Recently, a damage index specific for APS (DIAPS) was developed. There are, however, no data regarding the comparison of its performance and long-term damage in primary antiphospholipid syndrome (PAPS) and APS related to systemic lupus erythematosus (SLE; APS + SLE). The primary purpose of this study was therefore to compare the long-term damage in patients with these conditions.
This is a retrospective analysis of a single tertiary center cohort followed for approximately 10 years using a standardized prospective electronic chart database. Fifty consecutive PAPS patients age matched with 50 APS+SLE patients were consecutively selected for the study, and DIAPS was calculated once a year during follow-up. Long-term damage and damage kinetics in both groups were compared.
PAPS and APS + SLE had comparable age (47.10 ± 12.4 vs. 44.04 ± 10.80 years; = 0.19) and time of follow-up (9.40 ± 3.60 vs. 10.94 ± 4.50 years; = 0.06). At diagnosis, PAPS had higher DIAPS than APS + SLE (1.72 ± 1.17 vs. 0.82 ± 0.96; < 0.001). At the end of the 10-year follow-up, both groups presented comparable mean damage scores (2.04 ± 1.50 vs. 2.24 ± 1.61; = 0.52). The damage increment throughout the observation period for PAPS was solely 35%, whereas for APS + SLE it was gradual, persistent and reached 139% at the end of follow-up, with a total damage increment for PAPS lower than APS + SLE (0.43 ± 0.30 vs. 1.22 ± 1.24; < 0.001). Of note, the frequency of individuals who acquired damage was lower in PAPS than in APS + SLE (32% vs. 71%; < 0.001). PAPS also had a longer delay in diagnosis than APS + SLE (4.00 ± 4.20 vs. 2.54 ± 3.05 years; = 0.04). This delay was positively correlated with a higher damage score at diagnosis ( = 0.36, < 0.001) in all groups.
We identified a distinct pattern of damage in PAPS and APS related to SLE. Damage in PAPS is an early event, while APS+SLE is associated with higher long-term damage, with a striking increment of damage along the follow-up. A diagnosis delay is correlated with higher damage scores. Damage surveillance therefore requires different approaches for these two conditions.
抗磷脂综合征（APS）是一种获得性血栓形成倾向，影响年轻的有生育能力的个体，会对生活质量造成永久性损害和负面影响。最近，开发了一种针对 APS 的特定损伤指数（DIAPS）。然而，在原发性抗磷脂综合征（PAPS）和与系统性红斑狼疮（SLE）相关的 APS（APS+SLE）中，关于其表现和长期损伤的比较尚无数据。因此，本研究的主要目的是比较这些情况下的长期损伤。
这是一项回顾性分析，对大约 10 年时间使用标准化前瞻性电子图表数据库的单中心三级队列进行了分析。连续选择 50 例 PAPS 患者和 50 例 APS+SLE 患者作为匹配对照，在随访期间每年计算一次 DIAPS。比较两组的长期损伤和损伤动力学。
PAPS 和 APS+SLE 的年龄（47.10±12.4 岁 vs. 44.04±10.80 岁； = 0.19）和随访时间（9.40±3.60 岁 vs. 10.94±4.50 岁； = 0.06）相似。在诊断时，PAPS 的 DIAPS 高于 APS+SLE（1.72±1.17 vs. 0.82±0.96； < 0.001）。在 10 年随访结束时，两组的平均损伤评分相似（2.04±1.50 vs. 2.24±1.61； = 0.52）。在整个观察期内，PAPS 的损伤增加仅为 35%，而 APS+SLE 的损伤则逐渐增加且持续存在，随访结束时达到 139%，PAPS 的总损伤增加低于 APS+SLE（0.43±0.30 vs. 1.22±1.24； < 0.001）。值得注意的是，PAPS 中获得损伤的个体频率低于 APS+SLE（32% vs. 71%； < 0.001）。PAPS 的诊断延迟也长于 APS+SLE（4.00±4.20 岁 vs. 2.54±3.05 岁； = 0.04）。这种延迟与所有组中诊断时更高的损伤评分呈正相关（ = 0.36， < 0.001）。
我们在 PAPS 和与 SLE 相关的 APS 中发现了一种不同的损伤模式。PAPS 的损伤是早期事件，而 APS+SLE 则与更高的长期损伤相关，随着随访的进行，损伤明显增加。诊断延迟与更高的损伤评分相关。因此，对这两种情况需要采用不同的损伤监测方法。
