Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Catalonia, Spain.
Semin Arthritis Rheum. 2013 Feb;42(4):417-23. doi: 10.1016/j.semarthrit.2012.05.005. Epub 2012 Jul 10.
To analyze the clinical and laboratory characteristics of patients with catastrophic antiphospholipid syndrome (APS) who suffer relapses.
We analyzed the Web site--based international registry of patients with catastrophic APS ("CAPS Registry") http://infmed.fcrb.es/es/web/caps and selected those cases that relapsed.
Relapses were reported in 9 of 282 (3.2%) patients with catastrophic APS. A total of 35 episodes of catastrophic APS were found: 6 patients presented 2 relapses, 2 patients suffered 3 relapses, and 1 patient developed 17 relapses. However, the last patient was not included in the statistical analysis because his clinical and immunologic characteristics were not fully described. Therefore, a total of 18 episodes were analyzed. In 9 (50%) episodes, a precipitating factor was identified. The most frequent precipitating factor, found in 5 (28%) episodes, was infection. Brain, kidney, heart, and lung were the most common organs involved. Laboratory features of microangiopathic hemolytic anemia (MHA) were present in 13 of 18 (72%) episodes (definitive in 9, corresponding to 4 patients, and probable in 4, corresponding to 2 patients). Three relapses did not present with features of MHA and in the remaining 2 these data were not reported. The mortality rate was 38%.
Although relapses are rare in patients with catastrophic APS, these results support the hypothesis that an association between MHA and relapsing of catastrophic APS could be present.
分析灾难性抗磷脂综合征（APS）患者复发的临床和实验室特征。
我们分析了基于网站的灾难性 APS 患者国际注册中心（“CAPS 注册中心”）http://infmed.fcrb.es/es/web/caps，并选择了复发的病例。
282 例灾难性 APS 患者中，有 9 例（3.2%）发生复发。共发现 35 例灾难性 APS 发作：6 例患者出现 2 次复发，2 例患者出现 3 次复发，1 例患者出现 17 次复发。然而，由于最后一名患者的临床和免疫特征未完全描述，因此未将其纳入统计分析。因此，共分析了 18 例。在 9 例（50%）发作中发现了一个诱发因素。最常见的诱发因素是感染，在 5 例（28%）发作中发现。大脑、肾脏、心脏和肺部是最常见的受累器官。微血管性溶血性贫血（MHA）的实验室特征存在于 18 例中的 13 例（72%）（明确诊断 9 例，对应 4 例；可能诊断 4 例，对应 2 例）。3 例复发时未出现 MHA 特征，其余 2 例未报告这些数据。死亡率为 38%。
尽管灾难性 APS 患者的复发罕见，但这些结果支持了 MHA 与灾难性 APS 复发之间存在关联的假说。
