肺癌沙尔科毒症:一个全面的回顾:过去到现在
John A Belperio1, Michael C Fishbein2, Fereidoun Abtin3
1The Division of Pulmonary and Critical Care Medicine, Clinical Immunology, and Allergy, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Journal of autoimmunity
|October 21, 2023
概括
Sarcoidosis 是一种多系统颗粒性疾病,五年死亡率为7%,通常是由于心肺衰竭. 治疗包括葡萄糖皮质类药物,免疫抑制剂和抗TNF药物,但复发率差异很大.
科学领域:
- 肺部医学 肺部医学
- 免疫学 免疫学 免疫学
- 类风湿病学 类风湿病学
背景情况:
- 麻症是一种无菌的,非死性粒状瘤性疾病,病因不明,影响多个器官,主要是肺和胸部淋巴结.
- 它在全球范围内影响了2-160/100,000人,五年死亡率约为7%,主要是由于晚期心肺衰竭.
研究的目的:
- 审查与型硬化症相关的临床结果,治疗策略和死亡率,特别是肺部型硬化症.
- 为了突出肺性沙尔科毒症的进展和相关的并发症,如肺高血压.
主要方法:
- 审查现有的关于肉病流行病学,临床分期和治疗结果的文献.
- 分析死亡率数据和影响疾病进展和复发率的因素.
主要成果:
- 到40%的患者患有肺沙尔科毒症进展到第四阶段的疾病,从而增加了前毛囊性肺高血压的风险 (高达70%).
- 具有显著纤维化或肺高血压的IV期疾病具有>40%的5年死亡风险.
- 口服葡萄皮质类药物是第一线治疗;甲醇,阿扎西奥普林 (第二线) 和抗TNF药物 (第三线) 用于耐火性或类固醇节约性管理.
结论:
- 沙丘病的死亡率很大,主要是由心肺复杂症引起的,特别是在晚期肺部疾病中.
- 有效的管理涉及逐步治疗方法,但复发率仍然是一个挑战,受疾病阶段和患者因素的影响.
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