一个更新的管理儿童系统性红斑狼疮。

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Trindade VC, Carneiro-Sampaio M, Bonfa E Silva CA

一个更新的管理儿童系统性红斑狼疮。

Paediatr药物。2021年7月,23 (4):331 - 347。doi: 10.1007 / s40272 - 021 - 00457 - z。Epub 2021 7月10。

PubMed ID
34244988 (在PubMed
]
文摘

儿童系统性红斑狼疮(cSLE)是一种多系统的原型,炎症、异构自身免疫状况。这种疾病的特点是同时或连续的器官和系统参与,不可预知的耀斑和高水平的发病率和死亡率。种族/民族背景、社会经济地位、药物成本,难以获得卫生保健,红斑狼疮和可怜的坚持似乎影响结果和治疗反应。在本文中,更新cSLE病人的管理。关于发病机理,许多潜在的药物研究的目标。然而,大多数儿科患者的治疗药物与动力不足研究的基础上,建议治疗共识指南,或案例系列。管理实践对cSLE病人包括疾病活动和累积损伤的评估分数,non-live常规接种疫苗,体育活动,和解决心理健康问题。抗疟药和糖皮质激素仍是最常见的药物用于治疗cSLE,和羟氯喹对几乎所有cSLE病人建议。疾病修饰治疗风湿病的药物(DMARDs)应该为每个病人标准化,根据疾病爆发和cSLE严重性。霉酚酸酯或静脉注射环磷酰胺建议诱导治疗狼疮肾炎类III和IV。钙调磷酸酶抑制剂(环孢霉素、他克莫司voclosporin)似乎cSLE狼疮肾炎患者的另一个不错的选择。 Regarding B-cell-targeting biologic agents, rituximab may be used for refractory lupus nephritis patients in combination with another DMARD, and belimumab was recently approved by the US Food and Drug Administration for cSLE treatment in children aged > 5 years. New therapies targeting CD20, such as atacicept and telitacicept, seem to be promising drugs for SLE patients. Anti-interferon therapies (sifalimumab and anifrolumab) have shown beneficial results in phase II randomized control trials in adult SLE patients, as have some Janus kinase inhibitors, and these could be alternative treatments for pediatric patients with severe interferon-mediated inflammatory disease in the future. In addition, strict control of proteinuria and blood pressure is required in cSLE, especially with angiotensin-converting enzyme inhibitor and angiotensin receptor blocker use.

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