儿科原发性抗磷脂综合征:新标准,新机遇。

IF 1.9 4区 医学 Q3 RHEUMATOLOGY
Lupus Pub Date : 2025-09-01 Epub Date: 2025-07-12 DOI:10.1177/09612033251361040
Alfonso Ragnar Torres Jimenez, Berenice Sanchez Jara, Adriana Ivonne Cespedes Cruz, Virginia Ramirez Nova, Alejandra Velazquez Cruz, Guadalupe Del Consuelo Cortina Olvera, Vilma Carolina Bekker Méndez, Francisco Xavier Guerra Castillo
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引用次数: 0

摘要

目的介绍根据2023年ACR/EULAR APS分类标准诊断为原发性抗磷脂综合征患者的临床、实验室和治疗特点。材料与方法回顾性研究于2013年1月至2025年1月在墨西哥La Raza国家医疗中心总医院根据2023年ACR/EULAR分类标准诊断为原发性抗磷脂综合征的18岁以下患者。收集临床表现、实验室和治疗资料。结果我们收集了40例患者的资料,其中女性22例,男性18例,平均诊断年龄12.8岁。确诊时间为15.4周。14例患者发生血栓形成。血小板减少症34例。自身免疫性溶血性贫血18例Fisher Evans综合征17例33例为Livedo, 2例为皮肤溃疡,15例为雷诺氏病。癫痫发作2例,舞蹈病1例。我们没有发现心脏瓣膜受累。肾受累4例。aPTT延长39例(97.5%),狼疮抗凝剂阳性39例,抗心磷脂IgG阳性32例,抗心磷脂IgM阳性18例,抗b2gpi IgG阳性17例,阳性15例,抗b2gpi IgM阳性12例,阳性4例。治疗包括抗凝13例,抗血小板29例,类固醇35例,免疫抑制剂33例,利妥昔单抗6例。一名患者死于肺泡出血。2023年ACR/EULAR APS标准通过纳入非血栓性标准改善了儿科患者的分类;然而,在儿童中常见的其他非血栓性表现应该被考虑在内。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Primary antiphospholipid syndrome in pediatrics: New criteria, new opportunities.

ObjectiveWe present the clinical, laboratory, and treatment characteristics of patients diagnosed with primary antiphospholipid syndrome according to the 2023 ACR/EULAR APS classification criteria.Material and methodsA retrospective study was conducted in patients under 18 years of age, diagnosed with primary antiphospholipid syndrome according to the 2023 ACR/EULAR classification criteria, at the General Hospital of the National Medical Center La Raza, Mexico, from January 2013 to January 2025. Data on clinical manifestations, laboratory, and treatment were collected.ResultsWe present data from 40 patients, 22 female and 18 male, with a mean age at diagnosis of 12.8 years. The time to diagnosis was 15.4 weeks. Thrombosis occurred in 14 patients. Thrombocytopenia in 34. Autoimmune hemolytic anemia in 18, Fisher Evans syndrome in 17. Livedo in 33, skin ulcers in 2, and Raynaud's phenomenon in 15. Epileptic seizures in 2 and chorea in 1. We did not find cardiac valvular involvement. Renal involvement was found in 4. Prolonged aPTT in 39 (97.5%), positive lupus anticoagulant in 39, positive anticardiolipin IgG in 32, positive anticardiolipin IgM in 18, anti-B2GPI IgG performed in 17 patients, positive in 15, anti-B2GPI IgM performed in 12, positive in 4. Treatment consisted of anticoagulation in 13 patients, antiplatelet agents in 29, steroids in 35 immunosuppressants in 33, and rituximab in 6. One patient died from an alveolar hemorrhage.ConclusionsThe 2023 ACR/EULAR APS criteria improve the classification of pediatric patients by including non-thrombotic criteria; however, there are other non-thrombotic manifestations that are common in children that should be taken into account in this population.

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来源期刊
Lupus
Lupus 医学-风湿病学
CiteScore
4.20
自引率
11.50%
发文量
225
审稿时长
1 months
期刊介绍: The only fully peer reviewed international journal devoted exclusively to lupus (and related disease) research. Lupus includes the most promising new clinical and laboratory-based studies from leading specialists in all lupus-related disciplines. Invaluable reading, with extended coverage, lupus-related disciplines include: Rheumatology, Dermatology, Immunology, Obstetrics, Psychiatry and Cardiovascular Research…
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