系统性红斑狼相关的急性肺部疾病
Félicien Triboulet1, Emmanuelle Guérin1,2, Samia Boussouar3
1Service de Médecine Interne 2, Centre de Référence National Lupus Systémique, Syndrome des Anticorps Anti-Phospholipides et Autres Maladies Auto-Immunes Systémiques Rares, Institut E3M, Assistance Publique-Hôpitaux de Paris (APHP), Hôpital La Pitié-Salpêtrière, Sorbonne Université, Paris, France.
系统性红斑狼相关的急性肺病是一种严重的疾病,通常在疾病发作时发生. 这项研究强调了其临床特征,治疗方法和有利的结果,低死亡率.
科学领域:
- 类风湿病学 类风湿病学
- 肺部病理学 肺部病理学
- 关键护理医学 关键护理医学
背景情况:
- 系统性红斑狼 (SLE) 是一种多系统的自身免疫性疾病.
- 与SLE相关的急性肺病 (ALD) 是一种罕见但严重的并发症.
- ALD可能导致急性呼吸衰竭,需要密集护理.
研究的目的:
- 描述与SLE相关的ALD的临床特征.
- 概述用于与SLE相关的ALD的治疗策略.
- 报告与SLE相关的ALD相关的结果和死亡率.
主要方法:
- 在1996年11月至2018年9月期间入院的SLE和ALD患者的回顾性研究.
- 排除急性肺损伤的其他原因,如感染和心力衰竭.
- 分析临床数据,成像 (胸部CT),支气管支气管洗 (BAL) 发现,治疗方法和患者的结果.
主要成果:
- 确定了14名患有16次ALD的患者;70%的病例是SLE的初始表现.
- 常见的发现包括基底整合和CT上的地面玻璃不透明,在BAL上有中性膜炎和膜出血.
- 大多数患者需要重症监护,治疗包括皮质类固醇,环胺和机械通风.
结论:
- 与SLE相关的急性呼吸衰竭是一种严重的表现,通常在SLE发作时出现.
- 在CT上基底整合和在BAL上膜出血是特征性发现.
- 虽然严重,但这个队列中的死亡率低于以前报告的,这需要在更大的研究中进一步调查.
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