关键词: Catastrophic antiphospholipid syndrome Mexico México Síndrome antifosfolípido catastrófico Triple terapia Triple therapy

来  源:   DOI:10.1016/j.reumae.2023.04.003

Abstract:
Catastrophic antiphospholipid syndrome (CAPS) is a rare entity, approximately 600 cases have been reported around the world, and the prevalence in Mexico is unknown.
OBJECTIVE: To determine the estimated prevalence of CAPS in Mexico.
METHODS: A literature search of isolated clinical cases or case series was conducted in diverse search engines, using the terms: \"Catastrophic Antiphospholipid Syndrome\" and \"Mexico\" in May 2022.
RESULTS: We found a series of retrospective cases in autopsies that included 12 cases, two reports that included 2 cases each, and reports of 11 isolated clinical cases; these publications were generated between 2003 and 2020. In total, we collected data on 27 cases of CAPS, of which 16 correspond to primary antiphospholipid syndrome, 10 are associated with systemic lupus erythematosus, and 1 case corresponds to systemic sclerosis. The estimated prevalence rate in the Mexican population in 2022 is 2 cases per 10,000,000 inhabitants. The estimated mortality was 68% in this case series.
CONCLUSIONS: Cases of catastrophic antiphospholipid syndrome in Mexico are underreported; identifying them will help improve current diagnostic and therapeutic strategies used in the country, encouraging the implementation of triple therapy and, in refractory cases, the use of eculizumab, to reduce current mortality.
摘要:
灾难性抗磷脂综合征(CAPS)是一种罕见的实体,世界各地报告了大约600例病例,墨西哥的患病率未知。
目的:确定墨西哥CAPS的估计患病率。
方法:在不同的搜索引擎中对孤立的临床病例或病例系列进行了文献检索,使用以下术语:“灾难性抗磷脂综合征”和“墨西哥”,2022年5月。
结果:我们在尸检中发现了一系列回顾性病例,其中包括12例,两份报告,各包括2例,以及11例孤立临床病例的报告;这些出版物产生于2003年至2020年之间。总的来说,我们收集了27例CAPS的数据,其中16对应于原发性抗磷脂综合征,10与系统性红斑狼疮有关,1例对应于系统性硬化症。2022年墨西哥人口的估计患病率为每1000万居民2例。在该病例系列中,估计死亡率为68%。
结论:墨西哥的灾难性抗磷脂综合征病例报告不足;识别它们将有助于改善该国目前使用的诊断和治疗策略,鼓励实施三联疗法,在难治性病例中,使用依库珠单抗,降低目前的死亡率。
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